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Major prion protein

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687: 1975: 664: 586: 561: 6119: 6104: 6089: 5984: 5744: 945: 938: 6149: 6134: 5909: 693: 592: 5924: 6179: 5939: 5774: 5759: 6164: 6074: 5804: 5789: 6059: 6044: 5894: 5879: 5864: 5849: 5834: 5819: 6194: 5969: 5954: 5999: 2270:. (These ovine sites correspond to human sites 133, 151, and 168.) Polymorphisms of the PrP-VRQ form and PrP-ARQ form are associated with increased susceptibility, whereas PrP-ARR is associated with resistance. The National Scrapie Plan of the UK aims to breed out these scrapie polymorphisms by increasing the frequency of the resistant allele. However, PrP-ARR polymorphisms are susceptible to atypical scrapie, so this may prove unfruitful. 6029: 6014: 2570:(the building-blocks of proteins) in the prion protein. Others insert additional amino acids into the protein or cause an abnormally short protein to be made. These mutations cause the cell to make prion proteins with an abnormal structure. The abnormal protein PrP accumulates in the brain and destroys nerve cells, which leads to the mental and behavioral features of prion diseases. 38: 2468:
Modulation of signal transduction pathways has been demonstrated in cross-linking with antibodies and ligand-binding (hop/STI1 or copper). Given the diversity of interactions, effects, and distribution, PrP has been proposed as dynamic surface protein functioning in signaling pathways. Specific sites
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from PrP, but identical primary sequence. Whereas PrP has largely alpha helical and disordered domains, PrP has no alpha helix and an amyloid fibril core composed of a stack of PrP molecules glued together by parallel in-register intermolecular beta sheets. This refolding renders the PrP isoform
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The conversion of PrP to PrP conformation is the mechanism of transmission of fatal, neurodegenerative transmissible spongiform encephalopathies (TSE). This can arise from genetic factors, infection from external source, or spontaneously for reasons unknown. Accumulation of PrP corresponds with
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PrP is present in both the pre- and post-synaptic compartments, with the greatest concentration in the pre-synaptic portion. Considering this and PrP's suite of behavioral influences, the neural cell functions and interactions are of particular interest. Based on the copper ligand, one proposed
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In 2006 the production of cattle lacking PrP form of the major prion protein (PrP) protein was reported which were resistant to prion propagation with no apparent developmental abnormalities. Besides the study of bovine products free of prion proteins another use could be so that human
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models of Alzheimer's, attenuated the epilepsy-induced death phenotype seen in a subset of these animals. Taken collectively, recent evidence suggests PRNP may be important for conducing the neurotoxic effects of soluble Aβ-oligomers and the emergent disease state of Alzheimer's.
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mice to investigate deficiencies and differences. Initial attempts produced two strains of PrP-null mice that show no physiological or developmental differences when subjected to an array of tests. However, more recent strains have shown significant cognitive abnormalities.
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Giovagnoli AR, Di Fede G, Aresi A, Reati F, Rossi G, Tagliavini F (December 2008). "Atypical frontotemporal dementia as a new clinical phenotype of Gerstmann-Straussler-Scheinker disease with the PrP-P102L mutation. Description of a previously unreported Italian family".
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have been linked with cognitive impairment in the elderly as well as earlier cognitive decline. All of these studies investigated differences in codon 129, indicating its importance in the overall functionality of PrP, in particular with regard to memory.
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Robakis NK, Devine-Gage EA, Jenkins EC, Kascsak RJ, Brown WT, Krawczun MS, Silverman WP (October 1986). "Localization of a human gene homologous to the PrP gene on the p arm of chromosome 20 and detection of PrP-related antigens in normal human brain".
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Though present, very low levels of PrP exist in the liver and could be associated with liver fibrosis. Presence in the pituitary has been shown to affect neuroendocrine function in amphibians, but little is known concerning mammalian pituitary PrP.
2252:. Based on the progressive nature of spongiform encephalopathies, the predominant hypothesis posits that the change from normal PrP is caused by the presence and interaction with PrP. Strong support for this is taken from studies in which 2443:
PrP-null mice provide clues to a role in muscular physiology when subjected to a forced swimming test, which showed reduced locomotor activity. Aging mice with an overexpression of PRNP showed significant degradation of muscle tissue.
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has led to speculation on involvement in development. A wide range of studies has been conducted investigating the role in cell proliferation, differentiation, death, and survival. Engagement of PrP has been linked to activation of
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Further support for PrP's role in memory formation is derived from several population studies. A test of healthy young humans showed increased long-term memory ability associated with an MM or MV genotype when compared to VV.
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Though most attention is focused on PrP's presence in the nervous system, it is also abundant in immune system tissue. PrP immune cells include hematopoietic stem cells, mature lymphoid and myeloid compartments, and certain
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He J, Li X, Yang J, Huang J, Fu X, Zhang Y, Fan H (March 2013). "The association between the methionine/valine (M/V) polymorphism (rs1799990) in the PRNP gene and the risk of Alzheimer disease: an update by meta-analysis".
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Del Bo R, Comi GP, Giorda R, Crimi M, Locatelli F, Martinelli-Boneschi F, et al. (June 2003). "The 129 codon polymorphism of the prion protein gene influences earlier cognitive performance in Down syndrome subjects".
2759:. The decreased risk in V allele carriers is further limited to late-onset Alzheimer's disease only (≥ 65 years). PRNP can also functionally interact with polymorphisms in two other genes implicated in Alzheimer's, 2469:
along the protein bind other proteins, biomolecules, and metals. These interfaces allow specific sets of cells to communicate based on level of expression and the surrounding microenvironment. The anchoring on a
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PrP is highly conserved through mammals, lending credence to application of conclusions from test animals such as mice. Comparison between primates is especially similar, ranging from 92.9 to 99.6% similarity in
2607:– TSE in humans, transmitted via funerary cannibalism. Generally, affected family members were given, by tradition, parts of the central nervous system according to ritual when consuming deceased family members. 700: 599: 5062:
Collins S, McLean CA, Masters CL (September 2001). "Gerstmann-Sträussler-Scheinker syndrome,fatal familial insomnia, and kuru: a review of these less common human transmissible spongiform encephalopathies".
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Criado JR, Sánchez-Alavez M, Conti B, Giacchino JL, Wills DN, Henriksen SJ, et al. (2005). "Mice devoid of prion protein have cognitive deficits that are rescued by reconstitution of PrP in neurons".
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Roesler R, Walz R, Quevedo J, de-Paris F, Zanata SM, Graner E, et al. (August 1999). "Normal inhibitory avoidance learning and anxiety, but increased locomotor activity in mice devoid of PrP(C)".
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Croes EA, Dermaut B, Houwing-Duistermaat JJ, Van den Broeck M, Cruts M, Breteler MM, et al. (August 2003). "Early cognitive decline is associated with prion protein codon 129 polymorphism".
2256:-knockout mice are resistant to the introduction of PrP. Despite widespread acceptance of the conformation conversion hypothesis, some studies mitigate claims for a direct link between PrP and 4240:
Cagampang FR, Whatley SA, Mitchell AL, Powell JF, Campbell IC, Coen CW (1999). "Circadian regulation of prion protein messenger RNA in the rat forebrain: a widespread and synchronous rhythm".
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Kardos J, Kovács I, Hajós F, Kálmán M, Simonyi M (August 1989). "Nerve endings from rat brain tissue release copper upon depolarization. A possible role in regulating neuronal excitability".
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Kachiwala SJ, Harris SE, Wright AF, Hayward C, Starr JM, Whalley LJ, Deary IJ (September 2005). "Genetic influences on oxidative stress and their association with normal cognitive ageing".
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Berr C, Richard F, Dufouil C, Amant C, Alperovitch A, Amouyel P (September 1998). "Polymorphism of the prion protein is associated with cognitive impairment in the elderly: the EVA study".
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are confirmed PrP ligands that bind to its octarepeat region. Ligand binding causes a conformational change with unknown effect. Heavy metal binding at PrP has been linked to resistance to
1966:(vCJD). Similarities exist between kuru, thought to be due to human ingestion of diseased individuals, and vCJD, thought to be due to human ingestion of BSE-tainted cattle products. 3324:
Chesebro B, Trifilo M, Race R, Meade-White K, Teng C, LaCasse R, et al. (June 2005). "Anchorless prion protein results in infectious amyloid disease without clinical scrapie".
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at codon 178, which corroborates PrP's involvement in sleep-wake cycles. In addition, circadian regulation has been demonstrated in PrP mRNA, which cycles regularly with day-night.
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Nico PB, de-Paris F, VinadĂ© ER, Amaral OB, Rockenbach I, Soares BL, et al. (July 2005). "Altered behavioural response to acute stress in mice lacking cellular prion protein".
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Coitinho AS, Freitas AR, Lopes MH, Hajj GN, Roesler R, Walz R, et al. (December 2006). "The interaction between prion protein and laminin modulates memory consolidation".
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Kraus A, Hoyt F, Schwartz CL, Hansen B, Artikis E, Hughson AG, et al. (November 2021). "High-resolution structure and strain comparison of infectious mammalian prions".
2601:(mad-cow disease) – fatal neurodegenerative disease in cows, which can be transmitted to humans by ingestion of brain, spinal, or digestive tract tissue of an infected cow 6118: 6103: 6088: 7095: 5983: 2040:, and this proves to be integral to the transmission of conformational change; secreted PrP lacking the anchor component is unaffected by the infectious isoform. 4070:
Katamine S, Nishida N, Sugimoto T, Noda T, Sakaguchi S, Shigematsu K, et al. (December 1998). "Impaired motor coordination in mice lacking prion protein".
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Sandberg MK, Al-Doujaily H, Sharps B, Clarke AR, Collinge J (February 2011). "Prion propagation and toxicity in vivo occur in two distinct mechanistic phases".
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gene (called polymorphisms) do not cause prion diseases but may affect a person's risk of developing these diseases or alter the course of the disorders. An
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The strong association to neurodegenerative diseases raises many questions of the function of PrP in the brain. A common approach is using PrP-knockout and
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Liao YC, Lebo RV, Clawson GA, Smuckler EA (July 1986). "Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications".
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pharmaceuticals can be made in their blood without the danger that those products might get contaminated with the infectious agent that causes mad cow.
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Kovács GG, Trabattoni G, Hainfellner JA, Ironside JW, Knight RS, Budka H (November 2002). "Mutations of the prion protein gene phenotypic spectrum".
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Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ (August 1986). "Molecular cloning of a human prion protein cDNA".
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copper into cells from the surrounding environment. Researchers have also proposed roles for PrP in cell signaling or in the formation of
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results in decreased motor coordination. However, this effect is not a direct result of PrP's absence, and rather arises from increased
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outgrowth. The PrP-activated signal transduction pathway is associated with axon and dendritic outgrowth with a series of kinases.
1904: 7658: 2490: 2432: 5908: 3575:"Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro" 7646: 7424: 2391:(LTP). This effect can be positive or negative and is due to changes in neuronal excitability and synaptic transmission in the 5923: 8259: 7710: 7705: 7651: 7431: 5723: 2664:-knockout mice were found to be indistinguishable from control with respect to both neuronal death rates and measurements of 2303:
gene expression. Other observed differences include reduced stress response and increased exploration of novel environments.
1963: 2876: 23:, infectious forms of proteins which have so far been observed in almost all instances to be forms of PRNP, but need not be. 8264: 7164: 7090: 6220: 4782:"The persistence of long-term memory: a molecular approach to self-sustaining changes in learning-induced synaptic growth" 8193: 8188: 8183: 8178: 7105: 2855: 2708:
deletion has been prophylactically implemented by the cattle industry without apparent harm. In mice, this same deletion
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Linden R, Martins VR, Prado MA, Cammarota M, Izquierdo I, Brentani RR (April 2008). "Physiology of the prion protein".
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Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB (January 1995). "Prion protein gene variation among primates".
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activation is accompanied by a strong up-regulation of PrP, though it is not requisite. The lack of immunoresponse to
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mechanism, a calcium modulator, or a sensor for copper or oxidative stress. Loss of PrP function has been linked to
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activity, contributing to conflicting results when examining Alzheimer's survival rates. Of note, the deletion of
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carriers (VV and MV) show a 13% decreased risk with respect to developing Alzheimer's compared to the methionine
2048: 663: 2063:- terminal ends are removed posttranslationally, resulting in a mature length of 208 amino acids. For human and 7601: 7137: 5893: 5878: 5863: 5848: 5833: 5818: 4984:
Castilla J, Hetz C, Soto C (June 2004). "Molecular mechanisms of neurotoxicity of pathological prion protein".
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when injected with Aβ oligomers. In mammals, the full functional significance of PRNP remains unclear, as
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Americo TA, Chiarini LB, Linden R (June 2007). "Signaling induced by hop/STI-1 depends on endocytosis".
3817:"Cryo-EM structure of anchorless RML prion reveals variations in shared motifs between distinct strains" 3485:"Prion protein prevents heavy metals overloading of cells and thus protects them against their toxicity" 7822: 6247: 5998: 2209: 2103: 1865: 699: 598: 5631:"Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection" 5185:"Brain disease 'resistance gene' evolves in Papua New Guinea community; could offer insights into CJD" 6688: 5716: 5545:
Richt JA, Kasinathan P, Hamir AN, Castilla J, Sathiyaseelan T, Vargas F, et al. (January 2007).
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Montagna P, Gambetti P, Cortelli P, Lugaresi E (March 2003). "Familial and sporadic fatal insomnia".
3387:"Pseudoknots in prion protein mRNAs confirmed by comparative sequence analysis and pattern searching" 2656:
or a site-specific relational significance. In the case of direct injection of Aβ oligomers into the
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The propagation of PrP is a topic of great interest, as its accumulation is a pathological cause of
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varies between Alzheimer's mouse lines, as hAPPJ20 mice and TgCRND8 mice show a slight increase in
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The mechanism for conformational conversion to the scrapie isoform is speculated to be an elusive
1744: 1728: 493:, ASCR, AltPrP, CD230, CJD, GSS, KURU, PRIP, PrP, PrP27-30, PrP33-35C, PrPc, p27-30, prion protein 7836: 6028: 6013: 5686: 5184: 4193:"Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene" 2780: 2542: 2310: 2204:
nitrogens from the 2nd and 3rd glycines in the repeat. The ability to bind copper is, therefore,
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Medori R, Tritschler HJ, LeBlanc A, Villare F, Manetto V, Chen HY, et al. (February 1992).
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Balducci C, Beeg M, Stravalaci M, Bastone A, Sclip A, Biasini E, et al. (February 2010).
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deficits. This would suggest either an unequal relation between PRNP and Aβ oligomer-mediated
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in animals has yielded several conflicting findings. When Aβ oligomers were injected into the
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Zanata SM, Lopes MH, Mercadante AF, Hajj GN, Chiarini LB, Nomizo R, et al. (July 2002).
5370:"Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers" 4880:"Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers" 4429:
Shorter J, Lindquist S (June 2005). "Prions as adaptive conduits of memory and inheritance".
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PrP is a conformational isoform of PrP, but this orientation tends to accumulate in compact,
1989: 574: 6127:: Ovine recombinant PrP(114-234), ARR variant in complex with the VRQ14 Fab fragment (IgG2a) 4472:
Papassotiropoulos A, Wollmer MA, Aguzzi A, Hock C, Nitsch RM, de Quervain DJ (August 2005).
8269: 7760: 7473: 6112:: Ovine recombinant PrP(114-234), VRQ variant in complex with the Fab of the VRQ14 antibody 6097:: Ovine recombinant PrP(114-234), ARQ variant in complex with the Fab of the VRQ14 antibody 5709: 5381: 5149:
Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Campbell T, et al. (November 2009).
4891: 4340: 3980: 3881: 3828: 3771: 3681: 3333: 3243: 3032: 2977: 2755:(MM). However, the protective effects of variant V carriers have been found exclusively in 2736: 2669: 2648:
deletion did not offer protection, only anti-PrP antibodies prevented long-term memory and
2478: 2435:(TSE), neurodegenerative diseases caused by prions, could stem from the tolerance for PrP. 2416: 2141: 5270:
Laurén J (2014). "Cellular prion protein as a therapeutic target in Alzheimer's disease".
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BĂĽeler H, Aguzzi A, Sailer A, Greiner RA, Autenried P, Aguet M, Weissmann C (July 1993).
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Sparkes RS, Simon M, Cohn VH, Fournier RE, Lem J, Klisak I, et al. (October 1986).
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Ross CA, Poirier MA (July 2004). "Protein aggregation and neurodegenerative disease".
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Some research indicates PrP involvement in neuronal development, differentiation, and
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Herms J, Tings T, Gall S, Madlung A, Giese A, Siebert H, et al. (October 1999).
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Hoyt F, Standke HG, Artikis E, Schwartz CL, Hansen B, Li K, et al. (July 2022).
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Manka SW, Zhang W, Wenborn A, Betts J, Joiner S, Saibil HR, et al. (July 2022).
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The precise function of PrP is not yet known. It may play a role in the transport of
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are composed of smaller Aβ plaques, and are the most damaging to the integrity of a
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repeats with sequence PHGGGWGQ (though the first repeat has the slightly modified,
2137: 2099: 2006: 779: 710: 654: 609: 5752:: PRION PROTEIN DOMAIN PRP(121-231) FROM MOUSE, NMR, 2 MINIMIZED AVERAGE STRUCTURE 5681: 2588:
In addition, some prion diseases can be transmitted from external sources of PrP.
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Zomosa-Signoret V, Arnaud JD, Fontes P, Alvarez-Martinez MT, Liautard JP (2008).
2966:"Assignment of the human and mouse prion protein genes to homologous chromosomes" 2701: 2060: 2029: 754: 538: 4208: 3120: 3103: 2916:
National Center for Biotechnology Information, U.S. National Library of Medicine
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National Center for Biotechnology Information, U.S. National Library of Medicine
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Proceedings of the National Academy of Sciences of the United States of America
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Proceedings of the National Academy of Sciences of the United States of America
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Proceedings of the National Academy of Sciences of the United States of America
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at sites 136, 154, and 171 are associated with varying susceptibility to ovine
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Laurén J, Gimbel DA, Nygaard HB, Gilbert JW, Strittmatter SM (February 2009).
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Hwang D, Lee IY, Yoo H, Gehlenborg N, Cho JH, Petritis B, et al. (2009).
5151:"A novel protective prion protein variant that colocalizes with kuru exposure" 5032: 4878:
Laurén J, Gimbel DA, Nygaard HB, Gilbert JW, Strittmatter SM (February 2009).
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Aguzzi A, Baumann F, Bremer J (2008). "The prion's elusive reason for being".
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179 of the second helix and Cys214 of the third helix (human PrP numbering).
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Kanaani J, Prusiner SB, Diacovo J, Baekkeskov S, Legname G (December 2005).
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Damberger FF, Christen B, PĂ©rez DR, Hornemann S, WĂĽthrich K (October 2011).
3044: 2990: 915: 793: 8138: 7446: 7436: 6967: 6933: 5664: 5646: 5615: 5580: 5497: 5450: 5411: 5349: 5291: 5239: 5176: 5127: 5084: 5076: 5040: 5005: 4970: 4921: 4856: 4807: 4723: 4664: 4621: 4535: 4499: 4450: 4407: 4372: 4305: 4261: 4177: 4134: 4056: 4015: 3988: 3901: 3858: 3801: 3744: 3643: 3600: 3551: 3510: 3501: 3484: 3466: 3420: 3402: 3363: 3302: 3275: 3216: 3188: 3164: 3129: 2942: 2747:(rs1799990) is most closely associated with Alzheimer's disease. Variant V 2617: 2257: 2021: 1900: 772: 551: 5167: 5150: 4758: 4578: 4226: 4091: 4047: 4030: 3950: 3701: 3310: 3088: 3052: 3009: 2950: 1833: 1828: 8148: 7797: 7575: 7550: 7249: 6871: 6797: 6271: 4570: 4490: 4473: 2821: 2697: 2689: 2657: 2412: 2392: 2384: 2242: 2010: 1608: 1589: 5393: 5221: 4903: 3893: 3526:"Antioxidant activity related to copper binding of native prion protein" 3156: 8078: 7802: 7483: 7224: 7100: 6733: 6728: 6723: 6578: 6362: 5283: 3665:
Riek R, Hornemann S, Wider G, Glockshuber R, WĂĽthrich K (August 1997).
2752: 2729: 2721: 2595:– fatal neurodegenerative disease in sheep, not transmissible to humans 2567: 2554: 2550: 2457: 2296: 2284: 2217: 2197: 2169: 2095: 2072: 2044: 2017: 2009:. The human protein structure consists of a globular domain with three 1399:
regulation of intracellular calcium activated chloride channel activity
671: 568: 518: 6157:: Solution structure of the sheep prion protein with polymorphism H168 4613: 2928: 459: 455: 451: 447: 443: 439: 435: 431: 427: 423: 419: 415: 411: 407: 403: 399: 395: 391: 387: 383: 379: 375: 371: 367: 363: 359: 355: 351: 347: 343: 339: 335: 331: 327: 323: 319: 315: 311: 307: 303: 299: 295: 291: 287: 283: 279: 275: 271: 267: 263: 259: 255: 251: 247: 243: 239: 235: 231: 227: 223: 219: 215: 211: 207: 203: 199: 195: 191: 187: 183: 179: 175: 171: 167: 163: 159: 155: 151: 147: 143: 139: 135: 131: 127: 123: 119: 115: 111: 107: 103: 99: 95: 91: 87: 83: 7917: 7912: 7907: 7897: 7892: 7887: 7882: 6142:: THE CRYSTAL STRUCTURE OF THE GLOBULAR DOMAIN OF SHEEP PRION PROTEIN 2713: 2424: 2420: 2194: 2189:-deficient sequence PQGGGGWGQ). This is thought to generate a copper- 2186: 2129: 2080: 1993: 1463: 738: 725: 637: 624: 526: 5562: 4688:"Evidence of presynaptic location and function of the prion protein" 4442: 2791:
in cases of ambiguously diagnosed dementia, as the various forms of
2361:
substitution have been linked to earlier cognitive decline. Several
2345:(LN) is pivotal in memory processing and is likely modulated by the 8223: 8123: 7937: 7922: 7125: 7120: 7028: 6718: 5018: 3635: 2792: 2625: 2229: 2084: 1888: 1880: 1817: 5697:
Susan Lindquist's Seminar: "The Surprising World of Prion Biology"
4471: 4282: 2835:
organizing protein; also called STI1 (Stress-induced protein 1)).
2779:
at least in part contributed to three separate patients' atypical
2632:. The precise mechanism of soluble Aβ oligomers directly inducing 1409:
negative regulation of amyloid precursor protein catabolic process
8173: 8168: 8163: 8063: 7877: 7867: 7694: 7673: 7668: 7636: 7441: 7361: 7356: 7351: 7346: 7341: 7259: 6952: 6792: 6787: 6782: 6777: 6772: 6243: 2592: 2562:
progression of neurodegeneration and is the proposed cause. Some
2537: 2533: 2399: 2346: 2342: 2267: 2182: 2157: 2076: 1919: 1869: 1575: 1530: 1448: 1444: 5105: 3572: 3065: 1249:
negative regulation of DNA-binding transcription factor activity
506: 8113: 8073: 8058: 8042: 8037: 8017: 8012: 7947: 7755: 7725: 7689: 7680: 7493: 7461: 7325: 7320: 7290: 7269: 7229: 7189: 7055: 7033: 6992: 6916: 6911: 6906: 6901: 6896: 6891: 6760: 6755: 6750: 6698: 6683: 6553: 6543: 6506: 6501: 6419: 6414: 3871: 3229: 2748: 2733: 2629: 2578: 2519: 2515: 2428: 2358: 2334: 2172: 2133: 2121: 2115: 1801: 1485: 953: 5701: 4239: 4190: 3323: 1414:
positive regulation of protein localization to plasma membrane
8238: 8213: 8133: 8128: 8118: 8108: 8093: 8088: 8083: 8068: 8022: 8007: 7972: 7957: 7952: 7942: 7862: 7857: 7852: 7831: 7827: 7817: 7730: 7631: 7626: 7585: 7535: 7530: 7525: 7520: 7505: 7498: 7478: 7466: 7409: 7404: 7399: 7389: 7384: 7377: 7315: 7305: 7300: 7295: 7285: 7264: 7254: 7219: 7214: 7209: 7204: 7199: 7154: 7149: 7115: 7080: 7075: 7070: 7060: 7048: 7043: 7023: 7018: 7013: 7008: 6962: 6854: 6849: 6668: 6652: 6645: 6640: 6635: 6630: 6625: 6620: 6595: 6573: 6568: 6461: 6387: 6382: 6372: 4937:"The role of the cellular prion protein in the immune system" 4831:"Common molecular mechanisms in explicit and implicit memory" 4326: 3664: 2832: 2828: 2760: 2740: 2581:
that codes for a PRNP variant, G127V, provides resistance to
2529: 2201: 2056: 1916: 1339:
calcium-mediated signaling using intracellular calcium source
20: 5544: 4642: 2696:
codes for the enzyme Fyn, FYN-knockout mice display neither
2668:. It was further found that Aβ-oligomers bind to PrP at the 2620:(Aβ) oligomers, which are canonically implicated in causing 2616:
PrP protein is one of several cellular receptors of soluble
1992:
between the end (terminus) of the arm and position 13, from
8233: 8228: 8218: 8208: 8203: 8198: 8153: 8143: 8103: 8032: 8002: 7997: 7992: 7987: 7982: 7977: 7932: 7902: 7807: 7775: 7770: 7750: 7740: 7735: 7720: 7715: 7616: 7611: 7580: 7560: 7555: 7545: 7540: 7419: 7372: 7332: 6987: 6982: 6977: 6972: 6957: 6948: 6943: 6938: 6923: 6881: 6876: 6866: 6861: 6844: 6839: 6834: 6829: 6824: 6819: 6814: 6809: 6804: 6740: 6713: 6708: 6703: 6678: 6673: 6610: 6605: 6600: 6590: 6585: 6538: 6533: 6528: 6518: 6513: 6496: 6491: 6486: 6476: 6471: 6456: 6451: 6446: 6436: 6431: 6426: 6409: 6404: 6399: 6350: 6318: 6313: 6308: 6291: 6286: 6281: 6276: 5474: 4069: 3444: 3385:
Barrette I, Poisson G, Gendron P, Major F (February 2001).
3384: 2383:. In this role, the protein could serve as either a copper 2337:, this is attributable to Doppel gene expression. However, 2300: 2125: 1943: 1857: 37: 5367: 4877: 4474:"The prion gene is associated with human long-term memory" 4155: 3814: 3288: 2036:(GPI) membrane anchor at the COOH-terminal tethers PrP to 7745: 6357: 6345: 6340: 6335: 6325: 6298: 6266: 5628: 4031:"PrP knock-out and PrP transgenic mice in prion research" 3922: 3478: 3476: 3104:"Shattuck lecture--neurodegenerative diseases and prions" 2693: 2692:, another protein implicated in Alzheimer's. As the gene 2677: 2153: 1229:
negative regulation of calcineurin-NFAT signaling cascade
5148: 4385: 4112: 2963: 2688:, forming a complex with Fyn and excessively activating 2102:), which is thought to be involved in regulation of PrP 1359:
positive regulation of peptidyl-tyrosine phosphorylation
1254:
negative regulation of T cell receptor signaling pathway
762: 4736: 4556: 3722: 3143:
Weissmann C (November 2004). "The state of the prion".
3022: 2783:
within the same family, suggesting a new phenotype for
2333:
consolidation deficits have been demonstrated. As with
2205: 1429:
regulation of calcium ion import across plasma membrane
1364:
positive regulation of protein tyrosine kinase activity
3760:"2.7 Ă… cryo-EM structure of ex vivo RML prion fibrils" 3757: 3473: 1876:
but occurs in many other tissues throughout the body.
1379:
negative regulation of long-term synaptic potentiation
1183:
anchored component of external side of plasma membrane
5593: 5203: 4685: 4512: 3482: 1264:
negative regulation of activated T cell proliferation
927: 5917:: HUMAN PRION PROTEIN (MUTANT E200K) FRAGMENT 90-231 5061: 4934: 4002:
Atkinson M (October 2001). "National scrapie plan".
2484: 2325:
While null mice exhibit normal learning ability and
2079:
PrP has glycosylation sites as Asn180 and Asn196. A
1369:
positive regulation of protein targeting to membrane
5596:
Biochemical and Biophysical Research Communications
3966: 3069:
Biochemical and Biophysical Research Communications
2499:gene have been identified in people with inherited 2313:is thought to be the result of a point mutation in 1309:
regulation of potassium ion transmembrane transport
5932:: HUMAN PRION PROTEIN MUTANT E200K FRAGMENT 90-231 5470: 5468: 4935:Isaacs JD, Jackson GS, Altmann DM (October 2006). 3232:"Cellular prion protein conformation and function" 3191:"Physiological role of the cellular prion protein" 2872: 2870: 2868: 2851: 2849: 2847: 1404:negative regulation of dendritic spine maintenance 1384:regulation of glutamate receptor signaling pathway 1219:negative regulation of interferon-gamma production 4828: 4028: 3523: 2147: 709: 608: 8251: 4983: 4428: 2438: 1294:negative regulation of interleukin-17 production 5465: 2865: 2844: 2684:. Soluble Aβ oligomers also bind to PrP at the 1354:regulation of peptidyl-tyrosine phosphorylation 1349:positive regulation of neuron apoptotic process 1304:negative regulation of interleukin-2 production 5363: 5361: 5359: 5265: 5263: 5261: 5259: 5257: 5255: 5253: 5251: 5249: 4779: 3524:Brown DR, Clive C, Haswell SJ (January 2001). 2877:GRCm38: Ensembl release 89: ENSMUSG00000079037 2379:function casts PrP as a copper buffer for the 1214:negative regulation of protein phosphorylation 1057:type 8 metabotropic glutamate receptor binding 1052:type 5 metabotropic glutamate receptor binding 6228: 5717: 5427: 4780:Bailey CH, Kandel ER, Si K (September 2004). 3925:"Mice devoid of PrP are resistant to scrapie" 3483:PrÄŤina M, Kontseková E, Novák M (June 2015). 1394:negative regulation of amyloid-beta formation 5622: 5587: 5547:"Production of cattle lacking prion protein" 5309: 5307: 5305: 5303: 5301: 5197: 5099: 5055: 5012: 4977: 4928: 4871: 4822: 4773: 4679: 4636: 4593: 4506: 4465: 4422: 4379: 4320: 4276: 4233: 4149: 4106: 4022: 3865: 3615: 3517: 3317: 3223: 3136: 2767:, to compound risk for both Alzheimer's and 1907:version PrP is associated with a variety of 5356: 5246: 4829:Barco A, Bailey CH, Kandel ER (June 2006). 4730: 4550: 4184: 4063: 3916: 3621: 3282: 3095: 2856:GRCh38: Ensembl release 89: ENSG00000171867 2160:. PrP attaches to the outer surface of the 16:Protein involved in multiple prion diseases 6235: 6221: 5724: 5710: 5521:"Scientists Announce Mad Cow Breakthrough" 5423: 5421: 3962: 3960: 3440: 3438: 3436: 3434: 3432: 3430: 3184: 3182: 3059: 3016: 2957: 2922: 2223: 6252:list of human clusters of differentiation 6187:: Mouse Prion Protein with mutation N174T 5947:: HUMAN PRION PROTEIN 121-230 M166C/E221C 5689:at the U.S. National Library of Medicine 5654: 5570: 5401: 5339: 5298: 5229: 5166: 4960: 4911: 4846: 4797: 4713: 4703: 4489: 4362: 4352: 4216: 4046: 3940: 3848: 3791: 3590: 3541: 3500: 3410: 3345: 3265: 3255: 3206: 3142: 3119: 2999: 2989: 2636:is unknown, and experimental deletion of 2433:transmissible spongiform encephalopathies 1344:negative regulation of catalytic activity 1324:negative regulation of protein processing 5320:Intractable & Rare Diseases Research 4158:Brain Research. Molecular Brain Research 4001: 3101: 1988:gene is located on the short (p) arm of 1973: 1239:negative regulation of apoptotic process 1193:intracellular membrane-bounded organelle 5782:: BOVINE PRION PROTEIN FRAGMENT 121-230 5767:: BOVINE PRION PROTEIN FRAGMENT 121-230 5418: 5316:"Alzheimer's disease and prion protein" 5313: 3957: 3427: 3179: 2785:Gerstmann–Sträussler–Scheinker syndrome 2526:Gerstmann–Sträussler–Scheinker syndrome 2491:Transmissible spongiform encephalopathy 2373: 2291:As the null mice age, a marked loss of 1956:Gerstmann–Sträussler–Scheinker syndrome 8252: 6172:: mouse prion protein fragment 121-231 6082:: HUMAN PRION PROTEIN FRAGMENT 121-230 5812:: BOVINE PRION PROTEIN RESIDUES 23-230 5797:: BOVINE PRION PROTEIN RESIDUES 23-230 5269: 3981:10.1146/annurev.neuro.31.060407.125620 2720:in both APPswe and SEN1dE9, two other 2611: 2456:Varying expression of PrP through the 1853:) is encoded in the human body by the 6216: 6067:: HUMAN PRION PROTEIN FRAGMENT 90-230 6052:: HUMAN PRION PROTEIN FRAGMENT 90-230 5705: 5518: 5206:"A systems approach to prion disease" 2787:. The same study proposed sequencing 2566:mutations lead to a change in single 1334:activation of protein kinase activity 714: 675: 670: 613: 572: 567: 5431:Journal of the Neurological Sciences 4941:Clinical and Experimental Immunology 4388:The European Journal of Neuroscience 2193:via nitrogen atoms in the histidine 1899:(scrapie) and an isoform located in 5902:: HUMAN PRION PROTEIN VARIANT R220K 5887:: HUMAN PRION PROTEIN VARIANT R220K 5872:: HUMAN PRION PROTEIN VARIANT S170N 5857:: HUMAN PRION PROTEIN VARIANT S170N 5842:: HUMAN PRION PROTEIN VARIANT M166V 5827:: HUMAN PRION PROTEIN VARIANT M166V 5191:(Press release). November 21, 2009. 5155:The New England Journal of Medicine 4197:The New England Journal of Medicine 4072:Cellular and Molecular Neurobiology 3108:The New England Journal of Medicine 2043:The primary sequence of PrP is 253 1389:positive regulation of neuron death 13: 6202:: Ovine Prion Protein Variant R168 4705:10.1523/JNEUROSCI.19-20-08866.1999 2769:sporadic Creutzfeldt–Jakob disease 2212:shows copper binding results in a 2071:sites exist on helices 2 and 3 at 1996:4,615,068 to base pair 4,630,233. 1879:The protein can exist in multiple 1259:regulation of protein localization 1067:protein-containing complex binding 14: 8281: 5674: 2644:of a mouse model of Alzheimer's, 2485:Diseases caused by PrP misfolding 2278: 2109: 1964:variant Creutzfeldt–Jakob disease 1932:transmissible mink encephalopathy 1424:cellular response to amyloid-beta 1319:positive regulation of cell death 992:transmembrane transporter binding 6192: 6177: 6162: 6147: 6132: 6117: 6102: 6087: 6072: 6057: 6042: 6027: 6012: 5997: 5982: 5967: 5952: 5937: 5922: 5907: 5892: 5877: 5862: 5847: 5832: 5817: 5802: 5787: 5772: 5757: 5742: 5065:Journal of Clinical Neuroscience 4953:10.1111/j.1365-2249.2006.03194.x 4848:10.1111/j.1471-4159.2006.03870.x 4400:10.1111/j.1460-9568.2006.05156.x 4029:Weissmann C, Flechsig E (2003). 3592:10.1111/j.1471-4159.2005.03469.x 3543:10.1046/j.1471-4159.2001.00009.x 2672:, indirectly overactivating the 2599:Bovine spongiform encephalopathy 2415:; also, it has been detected in 2405: 1928:feline spongiform encephalopathy 1924:bovine spongiform encephalopathy 943: 936: 698: 691: 685: 662: 597: 590: 584: 559: 36: 5977:: HUMAN PRION PROTEIN AT PH 7.0 5962:: HUMAN PRION PROTEIN AT PH 7.0 5731: 5538: 5512: 5142: 3995: 3808: 3751: 3716: 3658: 3566: 3378: 2811: 2503:, which include the following: 2049:post-translational modification 1279:cellular response to copper ion 1274:cellular copper ion homeostasis 1158:extrinsic component of membrane 5272:Journal of Alzheimer's Disease 2904: 2886: 2495:More than 20 mutations in the 1936:exotic ungulate encephalopathy 1143:anchored component of membrane 1108:integral component of membrane 954:More reference expression data 916:More reference expression data 882:Region I of hippocampus proper 808:Region I of hippocampus proper 1: 5120:10.1016/S1474-4422(03)00323-5 4254:10.1016/S0306-4522(99)00092-5 4170:10.1016/S0169-328X(99)00193-X 3969:Annual Review of Neuroscience 3694:10.1016/S0014-5793(97)00920-4 2838: 2573:Several other changes in the 2439:Muscles, liver, and pituitary 2148:PrP (normal cellular) isoform 1915:diseases such as in animals: 1434:neuron projection maintenance 1002:ATP-dependent protein binding 683: 582: 8260:Genes on human chromosome 20 4799:10.1016/j.neuron.2004.09.017 4751:10.1016/0304-3940(89)90565-X 4657:10.1016/j.neulet.2005.05.067 3942:10.1016/0092-8674(93)90360-3 3737:10.1016/j.molcel.2021.08.011 3291:Journal of Molecular Biology 3145:Nature Reviews. Microbiology 3081:10.1016/0006-291X(86)90796-5 2557:is present at amino acid 129 2522:is present at amino acid 129 2166:glycosylphosphatidylinositol 1999: 1895:such as the disease-causing 1244:response to oxidative stress 1093:mitochondrial outer membrane 836:Epithelium of choroid plexus 7: 8265:Clusters of differentiation 6248:clusters of differentiation 6007:: HUMAN PRION PROTEIN 61-84 4692:The Journal of Neuroscience 4209:10.1056/NEJM199202133260704 3121:10.1056/NEJM200105173442006 2820:exists between PrP and the 2802: 2451: 2273: 1891:-resistant form designated 1872:is most predominant in the 1374:dendritic spine maintenance 1299:protein homooligomerization 1062:signaling receptor activity 866:nucleus of stria terminalis 10: 8286: 5608:10.1016/j.bbrc.2007.04.202 5519:Weiss R (1 January 2007). 5314:Zhou J, Liu B (May 2013). 4986:Current Molecular Medicine 4115:Behavioural Brain Research 3841:10.1038/s41467-022-30458-6 3784:10.1038/s41467-022-30457-7 3459:10.1152/physrev.00007.2007 2488: 1866:cluster of differentiation 812:retinal pigment epithelium 18: 8051: 7845: 7594: 7278: 7001: 6661: 6259: 5737: 5490:10.1007/s10072-008-1025-z 5443:10.1016/j.jns.2013.01.020 5332:10.5582/irdr.2013.v2.2.35 5210:Molecular Systems Biology 5033:10.1007/s00415-002-0896-9 4835:Journal of Neurochemistry 4528:10.1007/s00415-003-1057-5 4298:10.1016/j.nbd.2005.01.001 4127:10.1016/j.bbr.2005.02.003 3579:Journal of Neurochemistry 3530:Journal of Neurochemistry 2912:"Mouse PubMed Reference:" 2894:"Human PubMed Reference:" 2795:can prove challenging to 2702:dendritic spine shrinkage 2508:Creutzfeldt–Jakob disease 2479:extracellular scaffolding 2320: 2309:is altered in null mice. 2034:glycophosphatidylinositol 1948:Creutzfeldt–Jakob disease 1832: 1827: 1823: 1816: 1800: 1794:Chr 2: 131.75 – 131.78 Mb 1781: 1762: 1758: 1721: 1697: 1690: 1671: 1667: 1650: 1626: 1619: 1606: 1602: 1587: 1583: 1574: 1561: 1557: 1542: 1538: 1529: 1516: 1512: 1497: 1493: 1484: 1469: 1462: 1458: 1442: 1047:glycosaminoglycan binding 1022:identical protein binding 972: 968: 951: 935: 926: 913: 862: 853: 800: 791: 761: 753: 749: 732: 719: 682: 661: 652: 648: 631: 618: 581: 558: 549: 545: 500: 497: 487: 480: 475: 80: 75: 58: 53: 48: 44: 35: 30: 5691:Medical Subject Headings 4998:10.2174/1566524043360654 4478:Human Molecular Genetics 4431:Nature Reviews. Genetics 4035:British Medical Bulletin 3102:Prusiner SB (May 2001). 1926:(BSE, mad cow disease), 1868:230). Expression of the 1419:response to amyloid-beta 902:anterior amygdaloid area 19:Not to be confused with 4354:10.1073/pnas.0911829107 4286:Neurobiology of Disease 4084:10.1023/A:1020234321879 3356:10.1126/science.1110837 3257:10.1073/pnas.1106325108 3045:10.1126/science.3014653 2991:10.1073/pnas.83.19.7358 2797:differentially diagnose 2781:frontotemporal dementia 2543:fatal familial insomnia 2357:patients with a single 2311:Fatal familial insomnia 2241:extremely resistant to 1969: 1952:fatal familial insomnia 1940:chronic wasting disease 1329:protein destabilization 1224:response to cadmium ion 5077:10.1054/jocn.2001.0919 3502:10.4149/av_2015_02_179 3391:Nucleic Acids Research 3303:10.1006/jmbi.1994.0030 3208:10.1051/vetres:2007048 2943:10.1089/dna.1986.5.315 2528:– usually a change in 2477:supports claims of an 2389:long-term potentiation 1981: 1234:response to copper ion 6037:: HUMAN PRION PROTEIN 6022:: HUMAN PRION PROTEIN 5478:Neurological Sciences 5168:10.1056/NEJMoa0809716 5108:The Lancet. Neurology 4004:The Veterinary Record 3821:Nature Communications 3764:Nature Communications 3447:Physiological Reviews 2680:enzyme, resulting in 2224:PrP (scrapie) isoform 1977: 1787:Chr 20: 4.69 – 4.7 Mb 1148:extracellular exosome 1133:endoplasmic reticulum 575:Chromosome 20 (human) 5647:10.1093/emboj/cdf325 5551:Nature Biotechnology 5021:Journal of Neurology 4739:Neuroscience Letters 4645:Neuroscience Letters 4571:10.1212/wnl.51.3.734 4516:Journal of Neurology 3731:(21): 4540–4551.e6. 3630:(7 Suppl): S10–S17. 3403:10.1093/nar/29.3.753 2670:postsynaptic density 2549:-178 is replaced by 2514:-200 is replaced by 2417:natural killer cells 2374:Neurons and synapses 2142:heavy metal toxicity 2083:bond exists between 2007:amino acid sequences 1942:(CWD) which affects 1168:postsynaptic density 1037:amyloid-beta binding 816:orbitofrontal cortex 716:2 F2|2 64.07 cM 677:Chromosome 2 (mouse) 76:List of PDB id codes 49:Available structures 5687:PRNP+protein,+human 5680:PRNP (PrP) gene at 5525:The Washington Post 5394:10.1038/nature07761 5386:2009Natur.457.1128L 5380:(7233): 1128–1132. 5222:10.1038/msb.2009.10 4904:10.1038/nature07761 4896:2009Natur.457.1128L 4890:(7233): 1128–1132. 4602:Annals of Neurology 4345:2010PNAS..107.2295B 4048:10.1093/bmb/66.1.43 3894:10.1038/nature09768 3886:2011Natur.470..540S 3833:2022NatCo..13.4005H 3776:2022NatCo..13.4004M 3686:1997FEBSL.413..282R 3338:2005Sci...308.1435C 3332:(5727): 1435–1439. 3248:2011PNAS..10817308D 3242:(42): 17308–17313. 3195:Veterinary Research 3157:10.1038/nrmicro1025 3037:1986Sci...233..364L 2982:1986PNAS...83.7358S 2666:synaptic plasticity 2642:cerebral ventricles 2622:Alzheimer's disease 2612:Alzheimer's disease 2463:signal transduction 2104:protein translation 1909:cognitive disorders 1847:major prion protein 1077:cuprous ion binding 997:microtubule binding 828:trigeminal ganglion 5284:10.3233/JAD-130950 4491:10.1093/hmg/ddi228 2238:tertiary structure 2032:-terminal tail. A 2028:tail, and a short 1982: 1564:ENSMUSG00000079037 1269:learning or memory 1207:Biological process 1086:Cellular component 1072:cupric ion binding 1027:copper ion binding 980:Molecular function 8247: 8246: 6210: 6209: 5641:(13): 3307–3316. 5161:(21): 2056–2065. 5027:(11): 1567–1582. 4698:(20): 8866–8875. 4614:10.1002/ana.10658 4484:(15): 2241–2246. 4394:(11): 3255–3264. 3880:(7335): 540–542. 3114:(20): 1516–1526. 3031:(4761): 364–367. 2976:(19): 7358–7362. 2654:neurodegeneration 2327:short-term memory 2250:neurodegeneration 2200:and deprotonated 2013:and a two-strand 1946:; and in humans: 1913:neurodegenerative 1843: 1842: 1839: 1838: 1812: 1811: 1777: 1776: 1752: 1751: 1686: 1685: 1661: 1660: 1615: 1614: 1596: 1595: 1570: 1569: 1551: 1550: 1525: 1524: 1506: 1505: 1454: 1453: 1007:metal ion binding 987:chaperone binding 964: 963: 960: 959: 922: 921: 909: 908: 878:perirhinal cortex 874:entorhinal cortex 847: 846: 745: 744: 644: 643: 471: 470: 467: 466: 59:Ortholog search: 8277: 6237: 6230: 6223: 6214: 6213: 6196: 6181: 6166: 6151: 6136: 6121: 6106: 6091: 6076: 6061: 6046: 6031: 6016: 6001: 5986: 5971: 5956: 5941: 5926: 5911: 5896: 5881: 5866: 5851: 5836: 5821: 5806: 5791: 5776: 5761: 5746: 5726: 5719: 5712: 5703: 5702: 5669: 5668: 5658: 5635:The EMBO Journal 5626: 5620: 5619: 5591: 5585: 5584: 5574: 5542: 5536: 5535: 5533: 5531: 5516: 5510: 5509: 5472: 5463: 5462: 5425: 5416: 5415: 5405: 5365: 5354: 5353: 5343: 5311: 5296: 5295: 5267: 5244: 5243: 5233: 5201: 5195: 5192: 5180: 5170: 5146: 5140: 5139: 5103: 5097: 5096: 5059: 5053: 5052: 5016: 5010: 5009: 4981: 4975: 4974: 4964: 4932: 4926: 4925: 4915: 4875: 4869: 4868: 4850: 4841:(6): 1520–1533. 4826: 4820: 4819: 4801: 4777: 4771: 4770: 4734: 4728: 4727: 4717: 4707: 4683: 4677: 4676: 4640: 4634: 4633: 4597: 4591: 4590: 4554: 4548: 4547: 4510: 4504: 4503: 4493: 4469: 4463: 4462: 4426: 4420: 4419: 4383: 4377: 4376: 4366: 4356: 4339:(5): 2295–2300. 4324: 4318: 4317: 4292:(1–2): 255–265. 4280: 4274: 4273: 4248:(4): 1201–1204. 4237: 4231: 4230: 4220: 4188: 4182: 4181: 4153: 4147: 4146: 4110: 4104: 4103: 4067: 4061: 4060: 4050: 4026: 4020: 4019: 3999: 3993: 3992: 3964: 3955: 3954: 3944: 3935:(7): 1339–1347. 3920: 3914: 3913: 3869: 3863: 3862: 3852: 3812: 3806: 3805: 3795: 3755: 3749: 3748: 3720: 3714: 3713: 3671: 3662: 3656: 3655: 3619: 3613: 3612: 3594: 3585:(5): 1373–1386. 3570: 3564: 3563: 3545: 3521: 3515: 3514: 3504: 3480: 3471: 3470: 3442: 3425: 3424: 3414: 3382: 3376: 3375: 3349: 3321: 3315: 3314: 3286: 3280: 3279: 3269: 3259: 3227: 3221: 3220: 3210: 3186: 3177: 3176: 3140: 3134: 3133: 3123: 3099: 3093: 3092: 3063: 3057: 3056: 3020: 3014: 3013: 3003: 2993: 2961: 2955: 2954: 2926: 2920: 2919: 2908: 2902: 2901: 2890: 2884: 2874: 2863: 2853: 2775:on codon 102 of 2686:dendritic spines 2650:spatial learning 2349:PKA and ERK1/2. 2339:spatial learning 2331:long-term memory 2307:Circadian rhythm 2138:oxidative stress 2100:prion pseudoknot 2075:181 and Asn197. 2053:Signal sequences 1825: 1824: 1796: 1789: 1772: 1756: 1755: 1747: 1695: 1694: 1691:RefSeq (protein) 1681: 1665: 1664: 1656: 1624: 1623: 1600: 1599: 1581: 1580: 1555: 1554: 1536: 1535: 1510: 1509: 1491: 1490: 1460: 1459: 1314:long-term memory 1188:nuclear membrane 1042:protease binding 970: 969: 956: 947: 940: 933: 932: 918: 858: 856:Top expressed in 851: 850: 820:endothelial cell 804:Brodmann area 23 796: 794:Top expressed in 789: 788: 768: 767: 751: 750: 741: 728: 717: 702: 695: 689: 678: 666: 650: 649: 640: 627: 616: 601: 594: 588: 577: 563: 547: 546: 541: 539:PRNP - orthologs 492: 485: 462: 73: 72: 67: 46: 45: 40: 28: 27: 8285: 8284: 8280: 8279: 8278: 8276: 8275: 8274: 8250: 8249: 8248: 8243: 8047: 7841: 7590: 7274: 6997: 6657: 6255: 6241: 6211: 6206: 6203: 6197: 6188: 6182: 6173: 6167: 6158: 6152: 6143: 6137: 6128: 6122: 6113: 6107: 6098: 6092: 6083: 6077: 6068: 6062: 6053: 6047: 6038: 6032: 6023: 6017: 6008: 6002: 5993: 5987: 5978: 5972: 5963: 5957: 5948: 5942: 5933: 5927: 5918: 5912: 5903: 5897: 5888: 5882: 5873: 5867: 5858: 5852: 5843: 5837: 5828: 5822: 5813: 5807: 5798: 5792: 5783: 5777: 5768: 5762: 5753: 5747: 5733: 5730: 5677: 5672: 5627: 5623: 5592: 5588: 5563:10.1038/nbt1271 5543: 5539: 5529: 5527: 5517: 5513: 5473: 5466: 5426: 5419: 5366: 5357: 5312: 5299: 5268: 5247: 5202: 5198: 5183: 5147: 5143: 5104: 5100: 5060: 5056: 5017: 5013: 4982: 4978: 4933: 4929: 4876: 4872: 4827: 4823: 4778: 4774: 4735: 4731: 4684: 4680: 4641: 4637: 4598: 4594: 4555: 4551: 4511: 4507: 4470: 4466: 4443:10.1038/nrg1616 4427: 4423: 4384: 4380: 4325: 4321: 4281: 4277: 4238: 4234: 4189: 4185: 4154: 4150: 4111: 4107: 4068: 4064: 4027: 4023: 4000: 3996: 3965: 3958: 3921: 3917: 3870: 3866: 3813: 3809: 3756: 3752: 3721: 3717: 3669: 3663: 3659: 3624:Nature Medicine 3620: 3616: 3571: 3567: 3522: 3518: 3489:Acta Virologica 3481: 3474: 3443: 3428: 3383: 3379: 3322: 3318: 3287: 3283: 3228: 3224: 3187: 3180: 3151:(11): 861–871. 3141: 3137: 3100: 3096: 3064: 3060: 3021: 3017: 2962: 2958: 2927: 2923: 2910: 2909: 2905: 2892: 2891: 2887: 2875: 2866: 2854: 2845: 2841: 2814: 2805: 2728:In humans, the 2614: 2493: 2487: 2454: 2441: 2408: 2376: 2323: 2281: 2276: 2226: 2150: 2112: 2025: 2002: 1972: 1860:also known as 1834:View/Edit Mouse 1829:View/Edit Human 1792: 1785: 1782:Location (UCSC) 1768: 1764: 1743: 1739: 1735: 1731: 1727: 1723: 1717: 1713: 1709: 1705: 1701: 1677: 1673: 1652: 1646: 1642: 1638: 1634: 1630: 1545:ENSG00000171867 1438: 1202: 1123:plasma membrane 1118:Golgi apparatus 1081: 1017:protein binding 1012:tubulin binding 952: 942: 941: 914: 905: 900: 896: 894:cingulate gyrus 892: 888: 884: 880: 876: 872: 868: 854: 843: 838: 834: 830: 826: 822: 818: 814: 810: 806: 792: 736: 723: 715: 705: 704: 703: 696: 676: 653:Gene location ( 635: 622: 614: 604: 603: 602: 595: 573: 550:Gene location ( 501: 488: 481: 82: 60: 24: 17: 12: 11: 5: 8283: 8273: 8272: 8267: 8262: 8245: 8244: 8242: 8241: 8236: 8231: 8226: 8221: 8216: 8211: 8206: 8201: 8196: 8191: 8186: 8181: 8176: 8171: 8166: 8161: 8156: 8151: 8146: 8141: 8136: 8131: 8126: 8121: 8116: 8111: 8106: 8101: 8096: 8091: 8086: 8081: 8076: 8071: 8066: 8061: 8055: 8053: 8049: 8048: 8046: 8045: 8040: 8035: 8030: 8025: 8020: 8015: 8010: 8005: 8000: 7995: 7990: 7985: 7980: 7975: 7970: 7965: 7960: 7955: 7950: 7945: 7940: 7935: 7930: 7925: 7920: 7915: 7910: 7905: 7900: 7895: 7890: 7885: 7880: 7875: 7870: 7865: 7860: 7855: 7849: 7847: 7843: 7842: 7840: 7839: 7834: 7825: 7820: 7815: 7810: 7805: 7800: 7795: 7790: 7785: 7780: 7779: 7778: 7773: 7763: 7758: 7753: 7748: 7743: 7738: 7733: 7728: 7723: 7718: 7713: 7708: 7703: 7698: 7692: 7683: 7678: 7677: 7676: 7671: 7661: 7656: 7655: 7654: 7649: 7639: 7634: 7629: 7624: 7619: 7614: 7609: 7604: 7598: 7596: 7592: 7591: 7589: 7588: 7583: 7578: 7573: 7568: 7563: 7558: 7553: 7548: 7543: 7538: 7533: 7528: 7523: 7518: 7513: 7508: 7503: 7502: 7501: 7496: 7486: 7481: 7476: 7471: 7470: 7469: 7464: 7459: 7449: 7444: 7439: 7434: 7429: 7428: 7427: 7422: 7412: 7407: 7402: 7397: 7392: 7387: 7382: 7381: 7380: 7375: 7365: 7359: 7354: 7349: 7344: 7335: 7330: 7329: 7328: 7323: 7318: 7308: 7303: 7298: 7293: 7288: 7282: 7280: 7276: 7275: 7273: 7272: 7267: 7262: 7257: 7252: 7247: 7242: 7237: 7232: 7227: 7222: 7217: 7212: 7207: 7202: 7197: 7192: 7187: 7182: 7177: 7172: 7167: 7162: 7157: 7152: 7147: 7146: 7145: 7140: 7130: 7129: 7128: 7123: 7113: 7108: 7103: 7098: 7093: 7088: 7083: 7078: 7073: 7068: 7063: 7058: 7053: 7052: 7051: 7046: 7036: 7031: 7026: 7021: 7016: 7011: 7005: 7003: 6999: 6998: 6996: 6995: 6990: 6985: 6980: 6975: 6970: 6965: 6960: 6955: 6946: 6941: 6936: 6931: 6926: 6921: 6920: 6919: 6914: 6909: 6904: 6899: 6894: 6884: 6879: 6874: 6869: 6864: 6859: 6858: 6857: 6852: 6842: 6837: 6832: 6827: 6822: 6817: 6812: 6807: 6802: 6801: 6800: 6795: 6790: 6785: 6780: 6775: 6765: 6764: 6763: 6758: 6753: 6743: 6738: 6737: 6736: 6731: 6726: 6716: 6711: 6706: 6701: 6696: 6691: 6686: 6681: 6676: 6671: 6665: 6663: 6659: 6658: 6656: 6655: 6650: 6649: 6648: 6643: 6638: 6633: 6628: 6623: 6613: 6608: 6603: 6598: 6593: 6588: 6583: 6582: 6581: 6576: 6571: 6566: 6556: 6551: 6546: 6541: 6536: 6531: 6526: 6521: 6516: 6511: 6510: 6509: 6504: 6494: 6489: 6484: 6479: 6474: 6469: 6464: 6459: 6454: 6449: 6444: 6439: 6434: 6429: 6424: 6423: 6422: 6417: 6407: 6402: 6397: 6392: 6391: 6390: 6385: 6380: 6375: 6365: 6360: 6355: 6354: 6353: 6343: 6338: 6333: 6328: 6323: 6322: 6321: 6316: 6311: 6301: 6296: 6295: 6294: 6289: 6284: 6279: 6274: 6263: 6261: 6257: 6256: 6240: 6239: 6232: 6225: 6217: 6208: 6207: 6205: 6204: 6198: 6191: 6189: 6183: 6176: 6174: 6168: 6161: 6159: 6153: 6146: 6144: 6138: 6131: 6129: 6123: 6116: 6114: 6108: 6101: 6099: 6093: 6086: 6084: 6078: 6071: 6069: 6063: 6056: 6054: 6048: 6041: 6039: 6033: 6026: 6024: 6018: 6011: 6009: 6003: 5996: 5994: 5988: 5981: 5979: 5973: 5966: 5964: 5958: 5951: 5949: 5943: 5936: 5934: 5928: 5921: 5919: 5913: 5906: 5904: 5898: 5891: 5889: 5883: 5876: 5874: 5868: 5861: 5859: 5853: 5846: 5844: 5838: 5831: 5829: 5823: 5816: 5814: 5808: 5801: 5799: 5793: 5786: 5784: 5778: 5771: 5769: 5763: 5756: 5754: 5748: 5741: 5738: 5735: 5734: 5729: 5728: 5721: 5714: 5706: 5700: 5699: 5694: 5684: 5676: 5675:External links 5673: 5671: 5670: 5621: 5602:(2): 620–625. 5586: 5557:(1): 132–138. 5537: 5511: 5484:(6): 405–410. 5464: 5437:(1–2): 89–95. 5417: 5355: 5297: 5278:(2): 227–244. 5245: 5196: 5194: 5193: 5141: 5114:(3): 167–176. 5098: 5071:(5): 387–397. 5054: 5011: 4992:(4): 397–403. 4976: 4927: 4870: 4821: 4772: 4745:(2): 139–144. 4729: 4678: 4651:(2): 116–120. 4635: 4608:(2): 275–276. 4592: 4565:(3): 734–737. 4549: 4522:(6): 688–692. 4505: 4464: 4437:(6): 435–450. 4421: 4378: 4319: 4275: 4232: 4203:(7): 444–449. 4183: 4164:(2): 349–353. 4148: 4121:(2): 173–181. 4105: 4078:(6): 731–742. 4062: 4021: 3994: 3956: 3915: 3864: 3807: 3750: 3725:Molecular Cell 3715: 3680:(2): 282–288. 3657: 3636:10.1038/nm1066 3614: 3565: 3516: 3495:(2): 179–184. 3472: 3453:(2): 673–728. 3426: 3397:(3): 753–758. 3377: 3347:10.1.1.401.781 3316: 3297:(4): 362–374. 3281: 3222: 3178: 3135: 3094: 3075:(2): 758–765. 3058: 3015: 2956: 2937:(4): 315–324. 2921: 2903: 2885: 2864: 2842: 2840: 2837: 2813: 2810: 2804: 2801: 2773:point mutation 2710:phenotypically 2682:excitotoxicity 2613: 2610: 2609: 2608: 2602: 2596: 2559: 2558: 2540: 2523: 2501:prion diseases 2489:Main article: 2486: 2483: 2453: 2450: 2440: 2437: 2407: 2404: 2381:synaptic cleft 2375: 2372: 2322: 2319: 2293:Purkinje cells 2280: 2279:Nervous system 2277: 2275: 2272: 2225: 2222: 2216:change at the 2214:conformational 2191:binding domain 2181:contains five 2168:anchor at its 2149: 2146: 2111: 2110:Ligand-binding 2108: 2065:golden hamster 2038:cell membranes 2023: 2001: 1998: 1971: 1968: 1887:form, and the 1874:nervous system 1841: 1840: 1837: 1836: 1831: 1821: 1820: 1814: 1813: 1810: 1809: 1807: 1805: 1798: 1797: 1790: 1783: 1779: 1778: 1775: 1774: 1760: 1759: 1753: 1750: 1749: 1741:NP_001073592.1 1737:NP_001073591.1 1733:NP_001073590.1 1719: 1718: 1692: 1688: 1687: 1684: 1683: 1669: 1668: 1662: 1659: 1658: 1648: 1647: 1621: 1617: 1616: 1613: 1612: 1604: 1603: 1597: 1594: 1593: 1585: 1584: 1578: 1572: 1571: 1568: 1567: 1559: 1558: 1552: 1549: 1548: 1540: 1539: 1533: 1527: 1526: 1523: 1522: 1514: 1513: 1507: 1504: 1503: 1495: 1494: 1488: 1482: 1481: 1476: 1471: 1467: 1466: 1456: 1455: 1452: 1451: 1440: 1439: 1437: 1436: 1431: 1426: 1421: 1416: 1411: 1406: 1401: 1396: 1391: 1386: 1381: 1376: 1371: 1366: 1361: 1356: 1351: 1346: 1341: 1336: 1331: 1326: 1321: 1316: 1311: 1306: 1301: 1296: 1291: 1286: 1281: 1276: 1271: 1266: 1261: 1256: 1251: 1246: 1241: 1236: 1231: 1226: 1221: 1216: 1210: 1208: 1204: 1203: 1201: 1200: 1195: 1190: 1185: 1180: 1175: 1173:inclusion body 1170: 1165: 1160: 1155: 1150: 1145: 1140: 1135: 1130: 1125: 1120: 1115: 1110: 1105: 1100: 1095: 1089: 1087: 1083: 1082: 1080: 1079: 1074: 1069: 1064: 1059: 1054: 1049: 1044: 1039: 1034: 1029: 1024: 1019: 1014: 1009: 1004: 999: 994: 989: 983: 981: 977: 976: 966: 965: 962: 961: 958: 957: 949: 948: 930: 924: 923: 920: 919: 911: 910: 907: 906: 904: 903: 899: 895: 891: 887: 883: 879: 875: 871: 867: 863: 860: 859: 848: 845: 844: 842: 841: 837: 833: 829: 825: 824:spinal ganglia 821: 817: 813: 809: 805: 801: 798: 797: 785: 784: 776: 765: 759: 758: 755:RNA expression 747: 746: 743: 742: 734: 730: 729: 721: 718: 713: 707: 706: 697: 690: 684: 680: 679: 674: 668: 667: 659: 658: 646: 645: 642: 641: 633: 629: 628: 620: 617: 612: 606: 605: 596: 589: 583: 579: 578: 571: 565: 564: 556: 555: 543: 542: 499: 495: 494: 486: 478: 477: 473: 472: 469: 468: 465: 464: 78: 77: 69: 68: 57: 51: 50: 42: 41: 33: 32: 15: 9: 6: 4: 3: 2: 8282: 8271: 8268: 8266: 8263: 8261: 8258: 8257: 8255: 8240: 8237: 8235: 8232: 8230: 8227: 8225: 8222: 8220: 8217: 8215: 8212: 8210: 8207: 8205: 8202: 8200: 8197: 8195: 8192: 8190: 8187: 8185: 8182: 8180: 8177: 8175: 8172: 8170: 8167: 8165: 8162: 8160: 8157: 8155: 8152: 8150: 8147: 8145: 8142: 8140: 8137: 8135: 8132: 8130: 8127: 8125: 8122: 8120: 8117: 8115: 8112: 8110: 8107: 8105: 8102: 8100: 8097: 8095: 8092: 8090: 8087: 8085: 8082: 8080: 8077: 8075: 8072: 8070: 8067: 8065: 8062: 8060: 8057: 8056: 8054: 8050: 8044: 8041: 8039: 8036: 8034: 8031: 8029: 8026: 8024: 8021: 8019: 8016: 8014: 8011: 8009: 8006: 8004: 8001: 7999: 7996: 7994: 7991: 7989: 7986: 7984: 7981: 7979: 7976: 7974: 7971: 7969: 7966: 7964: 7961: 7959: 7956: 7954: 7951: 7949: 7946: 7944: 7941: 7939: 7936: 7934: 7931: 7929: 7926: 7924: 7921: 7919: 7916: 7914: 7911: 7909: 7906: 7904: 7901: 7899: 7896: 7894: 7891: 7889: 7886: 7884: 7881: 7879: 7876: 7874: 7871: 7869: 7866: 7864: 7861: 7859: 7856: 7854: 7851: 7850: 7848: 7844: 7838: 7835: 7833: 7829: 7826: 7824: 7821: 7819: 7816: 7814: 7811: 7809: 7806: 7804: 7801: 7799: 7796: 7794: 7791: 7789: 7786: 7784: 7781: 7777: 7774: 7772: 7769: 7768: 7767: 7764: 7762: 7759: 7757: 7754: 7752: 7749: 7747: 7744: 7742: 7739: 7737: 7734: 7732: 7729: 7727: 7724: 7722: 7719: 7717: 7714: 7712: 7709: 7707: 7704: 7702: 7699: 7696: 7693: 7691: 7687: 7684: 7682: 7679: 7675: 7672: 7670: 7667: 7666: 7665: 7662: 7660: 7657: 7653: 7650: 7648: 7645: 7644: 7643: 7640: 7638: 7635: 7633: 7630: 7628: 7625: 7623: 7620: 7618: 7615: 7613: 7610: 7608: 7605: 7603: 7600: 7599: 7597: 7593: 7587: 7584: 7582: 7579: 7577: 7574: 7572: 7569: 7567: 7564: 7562: 7559: 7557: 7554: 7552: 7549: 7547: 7544: 7542: 7539: 7537: 7534: 7532: 7529: 7527: 7524: 7522: 7519: 7517: 7514: 7512: 7509: 7507: 7504: 7500: 7497: 7495: 7492: 7491: 7490: 7487: 7485: 7482: 7480: 7477: 7475: 7472: 7468: 7465: 7463: 7460: 7458: 7455: 7454: 7453: 7450: 7448: 7445: 7443: 7440: 7438: 7435: 7433: 7430: 7426: 7423: 7421: 7418: 7417: 7416: 7413: 7411: 7408: 7406: 7403: 7401: 7398: 7396: 7393: 7391: 7388: 7386: 7383: 7379: 7376: 7374: 7371: 7370: 7369: 7366: 7363: 7360: 7358: 7355: 7353: 7350: 7348: 7345: 7343: 7339: 7336: 7334: 7331: 7327: 7324: 7322: 7319: 7317: 7314: 7313: 7312: 7309: 7307: 7304: 7302: 7299: 7297: 7294: 7292: 7289: 7287: 7284: 7283: 7281: 7277: 7271: 7268: 7266: 7263: 7261: 7258: 7256: 7253: 7251: 7248: 7246: 7243: 7241: 7238: 7236: 7233: 7231: 7228: 7226: 7223: 7221: 7218: 7216: 7213: 7211: 7208: 7206: 7203: 7201: 7198: 7196: 7193: 7191: 7188: 7186: 7183: 7181: 7178: 7176: 7173: 7171: 7168: 7166: 7163: 7161: 7158: 7156: 7153: 7151: 7148: 7144: 7141: 7139: 7136: 7135: 7134: 7131: 7127: 7124: 7122: 7119: 7118: 7117: 7114: 7112: 7109: 7107: 7104: 7102: 7099: 7097: 7094: 7092: 7089: 7087: 7084: 7082: 7079: 7077: 7074: 7072: 7069: 7067: 7064: 7062: 7059: 7057: 7054: 7050: 7047: 7045: 7042: 7041: 7040: 7037: 7035: 7032: 7030: 7027: 7025: 7022: 7020: 7017: 7015: 7012: 7010: 7007: 7006: 7004: 7000: 6994: 6991: 6989: 6986: 6984: 6981: 6979: 6976: 6974: 6971: 6969: 6966: 6964: 6961: 6959: 6956: 6954: 6950: 6947: 6945: 6942: 6940: 6937: 6935: 6932: 6930: 6927: 6925: 6922: 6918: 6915: 6913: 6910: 6908: 6905: 6903: 6900: 6898: 6895: 6893: 6890: 6889: 6888: 6885: 6883: 6880: 6878: 6875: 6873: 6870: 6868: 6865: 6863: 6860: 6856: 6853: 6851: 6848: 6847: 6846: 6843: 6841: 6838: 6836: 6833: 6831: 6828: 6826: 6823: 6821: 6818: 6816: 6813: 6811: 6808: 6806: 6803: 6799: 6796: 6794: 6791: 6789: 6786: 6784: 6781: 6779: 6776: 6774: 6771: 6770: 6769: 6766: 6762: 6759: 6757: 6754: 6752: 6749: 6748: 6747: 6744: 6742: 6739: 6735: 6732: 6730: 6727: 6725: 6722: 6721: 6720: 6717: 6715: 6712: 6710: 6707: 6705: 6702: 6700: 6697: 6695: 6692: 6690: 6687: 6685: 6682: 6680: 6677: 6675: 6672: 6670: 6667: 6666: 6664: 6660: 6654: 6651: 6647: 6644: 6642: 6639: 6637: 6634: 6632: 6629: 6627: 6624: 6622: 6619: 6618: 6617: 6614: 6612: 6609: 6607: 6604: 6602: 6599: 6597: 6594: 6592: 6589: 6587: 6584: 6580: 6577: 6575: 6572: 6570: 6567: 6565: 6562: 6561: 6560: 6557: 6555: 6552: 6550: 6547: 6545: 6542: 6540: 6537: 6535: 6532: 6530: 6527: 6525: 6522: 6520: 6517: 6515: 6512: 6508: 6505: 6503: 6500: 6499: 6498: 6495: 6493: 6490: 6488: 6485: 6483: 6480: 6478: 6475: 6473: 6470: 6468: 6465: 6463: 6460: 6458: 6455: 6453: 6450: 6448: 6445: 6443: 6440: 6438: 6435: 6433: 6430: 6428: 6425: 6421: 6418: 6416: 6413: 6412: 6411: 6408: 6406: 6403: 6401: 6398: 6396: 6393: 6389: 6386: 6384: 6381: 6379: 6376: 6374: 6371: 6370: 6369: 6366: 6364: 6361: 6359: 6356: 6352: 6349: 6348: 6347: 6344: 6342: 6339: 6337: 6334: 6332: 6329: 6327: 6324: 6320: 6317: 6315: 6312: 6310: 6307: 6306: 6305: 6302: 6300: 6297: 6293: 6290: 6288: 6285: 6283: 6280: 6278: 6275: 6273: 6270: 6269: 6268: 6265: 6264: 6262: 6258: 6253: 6249: 6245: 6238: 6233: 6231: 6226: 6224: 6219: 6218: 6215: 6201: 6195: 6190: 6186: 6180: 6175: 6171: 6165: 6160: 6156: 6150: 6145: 6141: 6135: 6130: 6126: 6120: 6115: 6111: 6105: 6100: 6096: 6090: 6085: 6081: 6075: 6070: 6066: 6060: 6055: 6051: 6045: 6040: 6036: 6030: 6025: 6021: 6015: 6010: 6006: 6000: 5995: 5991: 5985: 5980: 5976: 5970: 5965: 5961: 5955: 5950: 5946: 5940: 5935: 5931: 5925: 5920: 5916: 5910: 5905: 5901: 5895: 5890: 5886: 5880: 5875: 5871: 5865: 5860: 5856: 5850: 5845: 5841: 5835: 5830: 5826: 5820: 5815: 5811: 5805: 5800: 5796: 5790: 5785: 5781: 5775: 5770: 5766: 5760: 5755: 5751: 5745: 5740: 5739: 5736: 5727: 5722: 5720: 5715: 5713: 5708: 5707: 5704: 5698: 5695: 5692: 5688: 5685: 5683: 5679: 5678: 5666: 5662: 5657: 5652: 5648: 5644: 5640: 5636: 5632: 5625: 5617: 5613: 5609: 5605: 5601: 5597: 5590: 5582: 5578: 5573: 5568: 5564: 5560: 5556: 5552: 5548: 5541: 5526: 5522: 5515: 5507: 5503: 5499: 5495: 5491: 5487: 5483: 5479: 5471: 5469: 5460: 5456: 5452: 5448: 5444: 5440: 5436: 5432: 5424: 5422: 5413: 5409: 5404: 5399: 5395: 5391: 5387: 5383: 5379: 5375: 5371: 5364: 5362: 5360: 5351: 5347: 5342: 5337: 5333: 5329: 5325: 5321: 5317: 5310: 5308: 5306: 5304: 5302: 5293: 5289: 5285: 5281: 5277: 5273: 5266: 5264: 5262: 5260: 5258: 5256: 5254: 5252: 5250: 5241: 5237: 5232: 5227: 5223: 5219: 5215: 5211: 5207: 5200: 5190: 5186: 5182: 5181: 5178: 5174: 5169: 5164: 5160: 5156: 5152: 5145: 5137: 5133: 5129: 5125: 5121: 5117: 5113: 5109: 5102: 5094: 5090: 5086: 5082: 5078: 5074: 5070: 5066: 5058: 5050: 5046: 5042: 5038: 5034: 5030: 5026: 5022: 5015: 5007: 5003: 4999: 4995: 4991: 4987: 4980: 4972: 4968: 4963: 4958: 4954: 4950: 4946: 4942: 4938: 4931: 4923: 4919: 4914: 4909: 4905: 4901: 4897: 4893: 4889: 4885: 4881: 4874: 4866: 4862: 4858: 4854: 4849: 4844: 4840: 4836: 4832: 4825: 4817: 4813: 4809: 4805: 4800: 4795: 4791: 4787: 4783: 4776: 4768: 4764: 4760: 4756: 4752: 4748: 4744: 4740: 4733: 4725: 4721: 4716: 4711: 4706: 4701: 4697: 4693: 4689: 4682: 4674: 4670: 4666: 4662: 4658: 4654: 4650: 4646: 4639: 4631: 4627: 4623: 4619: 4615: 4611: 4607: 4603: 4596: 4588: 4584: 4580: 4576: 4572: 4568: 4564: 4560: 4553: 4545: 4541: 4537: 4533: 4529: 4525: 4521: 4517: 4509: 4501: 4497: 4492: 4487: 4483: 4479: 4475: 4468: 4460: 4456: 4452: 4448: 4444: 4440: 4436: 4432: 4425: 4417: 4413: 4409: 4405: 4401: 4397: 4393: 4389: 4382: 4374: 4370: 4365: 4360: 4355: 4350: 4346: 4342: 4338: 4334: 4330: 4323: 4315: 4311: 4307: 4303: 4299: 4295: 4291: 4287: 4279: 4271: 4267: 4263: 4259: 4255: 4251: 4247: 4243: 4236: 4228: 4224: 4219: 4214: 4210: 4206: 4202: 4198: 4194: 4187: 4179: 4175: 4171: 4167: 4163: 4159: 4152: 4144: 4140: 4136: 4132: 4128: 4124: 4120: 4116: 4109: 4101: 4097: 4093: 4089: 4085: 4081: 4077: 4073: 4066: 4058: 4054: 4049: 4044: 4040: 4036: 4032: 4025: 4017: 4013: 4009: 4005: 3998: 3990: 3986: 3982: 3978: 3974: 3970: 3963: 3961: 3952: 3948: 3943: 3938: 3934: 3930: 3926: 3919: 3911: 3907: 3903: 3899: 3895: 3891: 3887: 3883: 3879: 3875: 3868: 3860: 3856: 3851: 3846: 3842: 3838: 3834: 3830: 3826: 3822: 3818: 3811: 3803: 3799: 3794: 3789: 3785: 3781: 3777: 3773: 3769: 3765: 3761: 3754: 3746: 3742: 3738: 3734: 3730: 3726: 3719: 3711: 3707: 3703: 3699: 3695: 3691: 3687: 3683: 3679: 3675: 3668: 3661: 3653: 3649: 3645: 3641: 3637: 3633: 3629: 3625: 3618: 3610: 3606: 3602: 3598: 3593: 3588: 3584: 3580: 3576: 3569: 3561: 3557: 3553: 3549: 3544: 3539: 3535: 3531: 3527: 3520: 3512: 3508: 3503: 3498: 3494: 3490: 3486: 3479: 3477: 3468: 3464: 3460: 3456: 3452: 3448: 3441: 3439: 3437: 3435: 3433: 3431: 3422: 3418: 3413: 3408: 3404: 3400: 3396: 3392: 3388: 3381: 3373: 3369: 3365: 3361: 3357: 3353: 3348: 3343: 3339: 3335: 3331: 3327: 3320: 3312: 3308: 3304: 3300: 3296: 3292: 3285: 3277: 3273: 3268: 3263: 3258: 3253: 3249: 3245: 3241: 3237: 3233: 3226: 3218: 3214: 3209: 3204: 3200: 3196: 3192: 3185: 3183: 3174: 3170: 3166: 3162: 3158: 3154: 3150: 3146: 3139: 3131: 3127: 3122: 3117: 3113: 3109: 3105: 3098: 3090: 3086: 3082: 3078: 3074: 3070: 3062: 3054: 3050: 3046: 3042: 3038: 3034: 3030: 3026: 3019: 3011: 3007: 3002: 2997: 2992: 2987: 2983: 2979: 2975: 2971: 2967: 2960: 2952: 2948: 2944: 2940: 2936: 2932: 2925: 2917: 2913: 2907: 2899: 2895: 2889: 2882: 2878: 2873: 2871: 2869: 2861: 2857: 2852: 2850: 2848: 2843: 2836: 2834: 2830: 2826: 2823: 2819: 2809: 2800: 2798: 2794: 2790: 2786: 2782: 2778: 2774: 2770: 2766: 2762: 2758: 2754: 2750: 2746: 2742: 2738: 2735: 2731: 2726: 2723: 2719: 2715: 2711: 2707: 2703: 2699: 2695: 2691: 2687: 2683: 2679: 2675: 2674:NMDA receptor 2671: 2667: 2663: 2659: 2655: 2651: 2647: 2643: 2639: 2635: 2634:neurotoxicity 2631: 2627: 2623: 2619: 2606: 2603: 2600: 2597: 2594: 2591: 2590: 2589: 2586: 2584: 2580: 2576: 2571: 2569: 2565: 2556: 2552: 2548: 2547:aspartic acid 2544: 2541: 2539: 2535: 2531: 2527: 2524: 2521: 2517: 2513: 2512:glutamic acid 2509: 2506: 2505: 2504: 2502: 2498: 2492: 2482: 2480: 2476: 2475:lipid bilayer 2472: 2466: 2464: 2459: 2449: 2445: 2436: 2434: 2430: 2426: 2422: 2418: 2414: 2406:Immune system 2403: 2401: 2396: 2394: 2390: 2386: 2382: 2371: 2368: 2364: 2363:polymorphisms 2360: 2356: 2355:Down syndrome 2350: 2348: 2344: 2340: 2336: 2332: 2328: 2318: 2316: 2312: 2308: 2304: 2302: 2298: 2294: 2289: 2286: 2271: 2269: 2265: 2264:Polymorphisms 2261: 2259: 2255: 2251: 2246: 2244: 2239: 2235: 2231: 2221: 2219: 2215: 2211: 2207: 2203: 2199: 2196: 2192: 2188: 2184: 2180: 2179:Prion protein 2176: 2174: 2171: 2167: 2163: 2162:cell membrane 2159: 2155: 2145: 2143: 2140:arising from 2139: 2135: 2131: 2127: 2123: 2119: 2117: 2107: 2105: 2101: 2097: 2093: 2092:messenger RNA 2088: 2086: 2082: 2078: 2074: 2070: 2066: 2062: 2058: 2054: 2050: 2046: 2041: 2039: 2035: 2031: 2027: 2019: 2016: 2012: 2008: 1997: 1995: 1991: 1990:chromosome 20 1987: 1980: 1979:Chromosome 20 1976: 1967: 1965: 1961: 1957: 1953: 1949: 1945: 1941: 1937: 1933: 1929: 1925: 1921: 1918: 1914: 1910: 1906: 1902: 1898: 1894: 1890: 1886: 1883:: the normal 1882: 1877: 1875: 1871: 1867: 1863: 1859: 1856: 1852: 1848: 1835: 1830: 1826: 1822: 1819: 1815: 1808: 1806: 1803: 1799: 1795: 1791: 1788: 1784: 1780: 1773: 1771: 1767: 1761: 1757: 1754: 1748: 1746: 1742: 1738: 1734: 1730: 1726: 1720: 1716: 1712: 1708: 1704: 1700: 1696: 1693: 1689: 1682: 1680: 1676: 1670: 1666: 1663: 1657: 1655: 1649: 1645: 1641: 1637: 1633: 1629: 1625: 1622: 1620:RefSeq (mRNA) 1618: 1611: 1610: 1605: 1601: 1598: 1592: 1591: 1586: 1582: 1579: 1577: 1573: 1566: 1565: 1560: 1556: 1553: 1547: 1546: 1541: 1537: 1534: 1532: 1528: 1521: 1520: 1515: 1511: 1508: 1502: 1501: 1496: 1492: 1489: 1487: 1483: 1480: 1477: 1475: 1472: 1468: 1465: 1461: 1457: 1450: 1446: 1441: 1435: 1432: 1430: 1427: 1425: 1422: 1420: 1417: 1415: 1412: 1410: 1407: 1405: 1402: 1400: 1397: 1395: 1392: 1390: 1387: 1385: 1382: 1380: 1377: 1375: 1372: 1370: 1367: 1365: 1362: 1360: 1357: 1355: 1352: 1350: 1347: 1345: 1342: 1340: 1337: 1335: 1332: 1330: 1327: 1325: 1322: 1320: 1317: 1315: 1312: 1310: 1307: 1305: 1302: 1300: 1297: 1295: 1292: 1290: 1287: 1285: 1282: 1280: 1277: 1275: 1272: 1270: 1267: 1265: 1262: 1260: 1257: 1255: 1252: 1250: 1247: 1245: 1242: 1240: 1237: 1235: 1232: 1230: 1227: 1225: 1222: 1220: 1217: 1215: 1212: 1211: 1209: 1206: 1205: 1199: 1196: 1194: 1191: 1189: 1186: 1184: 1181: 1179: 1176: 1174: 1171: 1169: 1166: 1164: 1161: 1159: 1156: 1154: 1151: 1149: 1146: 1144: 1141: 1139: 1138:membrane raft 1136: 1134: 1131: 1129: 1126: 1124: 1121: 1119: 1116: 1114: 1111: 1109: 1106: 1104: 1103:mitochondrion 1101: 1099: 1096: 1094: 1091: 1090: 1088: 1085: 1084: 1078: 1075: 1073: 1070: 1068: 1065: 1063: 1060: 1058: 1055: 1053: 1050: 1048: 1045: 1043: 1040: 1038: 1035: 1033: 1032:lamin binding 1030: 1028: 1025: 1023: 1020: 1018: 1015: 1013: 1010: 1008: 1005: 1003: 1000: 998: 995: 993: 990: 988: 985: 984: 982: 979: 978: 975: 974:Gene ontology 971: 967: 955: 950: 946: 939: 934: 931: 929: 925: 917: 912: 901: 897: 893: 889: 885: 881: 877: 873: 869: 865: 864: 861: 857: 852: 849: 840:Pars compacta 839: 835: 831: 827: 823: 819: 815: 811: 807: 803: 802: 799: 795: 790: 787: 786: 783: 781: 777: 775: 774: 770: 769: 766: 764: 760: 756: 752: 748: 740: 735: 731: 727: 722: 712: 708: 701: 694: 688: 681: 673: 669: 665: 660: 656: 651: 647: 639: 634: 630: 626: 621: 611: 607: 600: 593: 587: 580: 576: 570: 566: 562: 557: 553: 548: 544: 540: 536: 532: 528: 524: 520: 516: 512: 508: 504: 496: 491: 484: 479: 474: 463: 461: 457: 453: 449: 445: 441: 437: 433: 429: 425: 421: 417: 413: 409: 405: 401: 397: 393: 389: 385: 381: 377: 373: 369: 365: 361: 357: 353: 349: 345: 341: 337: 333: 329: 325: 321: 317: 313: 309: 305: 301: 297: 293: 289: 285: 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Retrieved 5524: 5514: 5481: 5477: 5434: 5430: 5377: 5373: 5326:(2): 35–44. 5323: 5319: 5275: 5271: 5213: 5209: 5199: 5189:ScienceDaily 5188: 5158: 5154: 5144: 5111: 5107: 5101: 5068: 5064: 5057: 5024: 5020: 5014: 4989: 4985: 4979: 4944: 4940: 4930: 4887: 4883: 4873: 4838: 4834: 4824: 4792:(1): 49–57. 4789: 4785: 4775: 4742: 4738: 4732: 4695: 4691: 4681: 4648: 4644: 4638: 4605: 4601: 4595: 4562: 4558: 4552: 4519: 4515: 4508: 4481: 4477: 4467: 4434: 4430: 4424: 4391: 4387: 4381: 4336: 4332: 4322: 4289: 4285: 4278: 4245: 4242:Neuroscience 4241: 4235: 4200: 4196: 4186: 4161: 4157: 4151: 4118: 4114: 4108: 4075: 4071: 4065: 4038: 4034: 4024: 4007: 4003: 3997: 3972: 3968: 3932: 3928: 3918: 3877: 3873: 3867: 3824: 3820: 3810: 3767: 3763: 3753: 3728: 3724: 3718: 3677: 3674:FEBS Letters 3673: 3660: 3627: 3623: 3617: 3582: 3578: 3568: 3536:(1): 69–76. 3533: 3529: 3519: 3492: 3488: 3450: 3446: 3394: 3390: 3380: 3329: 3325: 3319: 3294: 3290: 3284: 3239: 3235: 3225: 3198: 3194: 3148: 3144: 3138: 3111: 3107: 3097: 3072: 3068: 3061: 3028: 3024: 3018: 2973: 2969: 2959: 2934: 2930: 2924: 2915: 2906: 2897: 2888: 2815: 2812:Interactions 2806: 2788: 2776: 2744: 2737:polymorphism 2727: 2717: 2705: 2661: 2645: 2637: 2618:amyloid beta 2615: 2587: 2574: 2572: 2563: 2560: 2496: 2494: 2467: 2455: 2446: 2442: 2409: 2397: 2377: 2366: 2351: 2324: 2314: 2305: 2290: 2282: 2262: 2258:cytotoxicity 2253: 2247: 2227: 2208:-dependent. 2177: 2151: 2120: 2113: 2089: 2069:glycosylated 2047:long before 2042: 2015:antiparallel 2003: 1985: 1983: 1901:mitochondria 1896: 1892: 1884: 1878: 1861: 1854: 1850: 1846: 1844: 1766:NP_001265185 1763: 1722: 1715:NP_001258490 1711:NP_001073592 1707:NP_001073591 1703:NP_001073590 1675:NM_001278256 1672: 1654:NM_001271561 1651: 1644:NM_001080123 1640:NM_001080122 1636:NM_001080121 1607: 1588: 1562: 1543: 1517: 1498: 1478: 1473: 1128:cell surface 778: 771: 737:131,780,349 724:131,751,848 498:External IDs 81: 25: 8270:Amyloidosis 5732:PDB gallery 4010:(15): 462. 3975:: 439–477. 3827:(1): 4005. 3770:(1): 4004. 2822:cochaperone 2818:interaction 2700:events nor 2698:excitotoxic 2658:hippocampus 2568:amino acids 2413:lymphocytes 2393:hippocampus 2385:homeostasis 2243:proteolysis 2198:side-chains 2183:octapeptide 2098:structure ( 2094:contains a 2045:amino acids 1745:NP_898902.1 1729:NP_000302.1 1198:postsynapse 476:Identifiers 8254:Categories 6250:(see also 5216:(1): 252. 4947:(1): 1–8. 2883:, May 2017 2862:, May 2017 2839:References 2757:Caucasians 2753:homozygote 2730:methionine 2722:transgenic 2555:methionine 2551:asparagine 2481:function. 2458:cell cycle 2297:cerebellum 2285:transgenic 2218:N-terminus 2170:C-terminal 2096:pseudoknot 1984:The human 1289:metabolism 1284:cell cycle 782:(ortholog) 636:4,701,590 623:4,686,350 519:HomoloGene 5530:1 January 4559:Neurology 4041:: 43–60. 3652:205383483 3342:CiteSeerX 2816:A strong 2714:epileptic 2626:oligomers 2532:102 from 2425:monocytes 2421:platelets 2234:secondary 2195:imidazole 2187:histidine 2130:manganese 2081:disulfide 2067:PrP, two 2026:-terminal 2011:α-helices 2000:Structure 1994:base pair 1905:misfolded 1770:NP_035300 1725:NP_898902 1699:NP_000302 1679:NM_011170 1632:NM_000311 1628:NM_183079 1464:Orthologs 1113:cytoplasm 898:subiculum 870:CA3 field 527:GeneCards 6244:Proteins 5682:GeneCard 5665:12093732 5616:17498662 5581:17195841 5506:20553167 5498:19030774 5459:31070331 5451:23399523 5412:19242475 5350:25343100 5292:23948943 5240:19308092 5177:19923577 5136:20822956 5128:12849238 5093:31976428 5085:11535002 5049:22688729 5041:12420099 5006:15354870 4971:16968391 4922:19242475 4865:26307975 4857:16805766 4808:15450159 4767:24917999 4724:10516306 4673:23642220 4665:16023289 4630:31538672 4622:12891686 4587:11352163 4544:21049364 4536:12796830 4500:15987701 4451:15931169 4416:17164351 4408:17156386 4373:20133875 4306:15837581 4270:42892475 4262:10391428 4178:10521590 4143:37511702 4135:15970215 4100:23409873 4057:14522848 4016:11688751 3989:18558863 3902:21350487 3859:35831291 3802:35831275 3745:34433091 3710:39791520 3644:15272267 3609:24329326 3601:16313516 3560:45647133 3552:11145979 3511:26104335 3467:18391177 3421:11160898 3372:10064966 3364:15933194 3276:21987789 3217:18073096 3201:(4): 9. 3173:20992257 3165:15494743 3130:11357156 2879:– 2858:– 2803:Research 2793:dementia 2676:via the 2624:. 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Index

prions

PDB
PDBe
RCSB
1E1G
1E1J
1E1P
1E1S
1E1U
1E1W
1FKC
1FO7
1H0L
1HJM
1HJN
1I4M
1OEH
1OEI
1QLX
1QLZ
1QM0
1QM1
1QM2
1QM3
2IV4
2IV5
2IV6
2K1D
2KUN

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