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Alpha-1 antitrypsin

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415: 392: 2499: 2476: 314: 289: 4659: 1806: 4539: 4704: 4689: 4674: 673: 666: 4629: 4599: 4584: 4554: 4614: 421: 320: 2588:(FDA) has approved the use of four alpha-1 antitrypsin products derived from a human plasma: Prolastin, Zemaira, Glassia, and Aralast. These products for intravenous augmentation A1AT therapy can cost up to $ 100,000 per year per patient. They are administered intravenously at a dose of 60 mg/kg once a week; higher doses do not provide additional benefit although they can be used in anticipation of an interruption of weekly administration, such as for a vacation. 4509: 4524: 4644: 42: 4494: 4569: 6160: 3688: 2592:(null, null), PiSZ). People are to be under optimal pharmacologic and non-pharmacologic treatment and show evidence of progressive lung disease (e.g. lower forced expiratory volume per second (FEV1) predicted, impaired walking capacity or increased number of exacerbations) as evaluated by a healthcare professional experienced in the treatment of alpha1-proteinase inhibitor deficiency. 1843:-antitrypsin (382 in the pre-processed form containing the 24 amino acid signal peptide), a residue essential for binding elastase; this is thought to be one of the primary mechanisms by which cigarette smoking (or second-hand smoke) can lead to emphysema. Because A1AT is expressed in the liver, certain mutations in the 2598:
Aerosolized-augmented A1AT therapy is under study. This involves inhaling purified human A1AT into the lungs and trapping the A1AT into the lower respiratory tract. However, inhaled A1AT may not reach the elastin fibers in the lung where elastase injury occurs. Further study is currently underway.
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Alpha1-proteinase inhibitor (Respreeza) was approved for medical use in the European Union in August 2015. It is indicated for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha1-proteinase inhibitor deficiency (e.g., genotypes PiZZ, PiZ (null), Pi
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A1AT not only binds to neutrophil elastase from inflammatory cells but also to elastase on the cell surface. In this latter role, elastase acts as a signaling molecule for cell movement, rather than as an enzyme. Besides liver cells, A1PI is also produced in bone marrow, lymphoid tissue, and the
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Besides limiting elastase activity to limit tissue degradation, A1PI also acts to induce locomotion of lymphocytes through tissue including immature T cells through the thymus where immature T cells mature to become immunocompetent T cells that are released into tissue to elevate immune
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Inactivation of A1AT by other enzymes during inflammation or infection can halt T cell migration precisely at the site of the pathological insult. This suggests that α1PI plays a key role in lymphocyte movement and immune surveillance, particularly in response to infection. A1AT is both
68: 1917:. This suggests that A1AT may play an anti-inflammatory or tissue-protecting role outside the lungs. These antibodies are associated with a more severe disease course, can be observed years before disease onset, and may predict the development of RA in 1980:, as it migrates toward the center of such an IEF gel. Other variants are less functional and are termed A-L and N-Z, dependent on whether they run proximal or distal to the M band. The presence of deviant bands on IEF can signify the presence of 3160:
Verheul MK, Yee A, Seaman A, Janssen GM, van Veelen PA, Drijfhout JW, et al. (June 2017). "Identification of carbamylated alpha 1 anti-trypsin (A1AT) as an antigenic target of anti-CarP antibodies in patients with rheumatoid arthritis".
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in which a deficiency of alpha-1 antitrypsin leads to a chronic uninhibited tissue breakdown. This causes the degradation especially of lung tissue and eventually leads to characteristic manifestations of
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with two different copies of the gene may have two different bands showing on electrofocusing, although heterozygote with one null mutant that abolishes expression of the gene will only show one band.
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Alkins SA, O'Malley P (March 2000). "Should health-care systems pay for replacement therapy in patients with alpha(1)-antitrypsin deficiency? A critical review and cost-effectiveness analysis".
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Owen MC, Brennan SO, Lewis JH, Carrell RW (September 1983). "Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder".
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The most common side effects include dizziness, headache, dyspnoea (shortness of breath) and nausea. Allergic reactions have been observed during treatment, some of which were severe.
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Kolarich D, Weber A, Turecek PL, Schwarz HP, Altmann F (June 2006). "Comprehensive glyco-proteomic analysis of human alpha1-antitrypsin and its charge isoforms".
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Carrell RW, Jeppsson JO, Laurell CB, Brennan SO, Owen MC, Vaughan L, et al. (July 1982). "Structure and variation of human alpha 1-antitrypsin".
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Kalsheker N, Morley S, Morgan K (April 2002). "Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin".
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to measure how much A1AT is present. Other detection methods include the use of enzyme-linked-immuno-sorbent-assays and radial immunodiffusion.
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Coakley RJ, Taggart C, O'Neill S, McElvaney NG (January 2001). "Alpha1-antitrypsin deficiency: biological answers to clinical questions".
3037:"Alpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk" 1742:. These enzymes are released locally in relatively low concentrations where they are immediately cleared by proteins such as A1AT. In the 5077: 4433: 3673: 65: 2172: 1937: 5734: 4613: 1259: 4415: 1252: 6047: 4141:
Lomas DA, Lourbakos A, Cumming SA, Belorgey D (April 2002). "Hypersensitive mousetraps, alpha1-antitrypsin deficiency and dementia".
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carry different amounts of negatively charged sialic acids; this causes the heterogeneity observed on normal A1AT when analysed by
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of 0.9–2.3 g/L (in the US the reference range is expressed as mg/dL or micromoles), but the concentration can rise manyfold upon
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Elliott PR, Abrahams JP, Lomas DA (January 1998). "Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation".
2570: 4508: 4439: 3086:"Oxidation of either methionine 351 or methionine 358 in alpha 1-antitrypsin causes loss of anti-neutrophil elastase activity" 6180: 4473: 1441: 4667:: Active site distortion is sufficient for proteinase inhibit second crystal structure of covalent serpin-proteinase complex 4523: 2680: 4812: 2770:"Acute-phase protein α1-anti-trypsin: diverting injurious innate and adaptive immune responses from non-authentic threats" 2659: 1628:
are most at risk for carrying one of the most common mutant forms of A1AT, the Z mutation (Glu342Lys on M1A, rs28929474).
6200: 5572: 4547:: A 2.1 ANGSTROM STRUCTURE OF AN UNCLEAVED ALPHA-1-ANTITRYPSIN SHOWS VARIABILITY OF THE REACTIVE CENTER AND OTHER LOOPS 414: 4981: 4923: 4861: 4807: 4802: 4744: 3205: 1969:(IEF) in the pH range 4.5-5.5, where the protein migrates in a gel according to its isoelectric point or charge in a 1473: 1508:
The protein was initially named "antitrypsin" because of its ability to bind and irreversibly inactivate the enzyme
1485: 3580: 2631:, another serpin that is analogous for protecting the body from excessive effects of its own inflammatory proteases 391: 3432: 5965: 5920: 5070: 4643: 3543: 2684: 2663: 2314: 2227: 2153: 1644:. However, one particular site shows a considerable amount of heterogeneity since tri- and even tetraantennary N- 1809:
Alpha-1 antitrypsin (white) with highlighted 'reactive centre loop' (blue) and A-sheet (light blue). (PDB: 1QLP)
6190: 6057: 5727: 5524: 3742:"Intravenous Alpha-1 Antitrypsin Therapy for Alpha-1 Antitrypsin Deficiency: The Current State of the Evidence" 3617: 3506: 3469: 2066: 1981: 1895: 1820: 1814: 1421: 1005: 3229: 6185: 5565: 313: 288: 3840:"Recombinant Alpha-1 Antitrypsin as Dry Powder for Pulmonary Administration: A Formulative Proof of Concept" 1449: 986: 982: 5255: 4493: 3623: 3586: 3549: 3512: 3475: 3438: 2585: 2494: 2387: 2021: 1720: 1469: 1358: 427: 326: 230: 2915: 2416: 5670: 2455: 1947:
The level of A1AT in serum is most often determined by adding an antibody that binds to A1AT, then using
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Kalsheker N (April 1989). "Alpha 1-antitrypsin: structure, function and molecular biology of the gene".
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region can be further divided into two sub-regions, termed "1" and "2". Alpha-1 antitrypsin is the main
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A1AT is a single-chain glycoprotein consisting of 394 amino acids in the mature form and exhibits many
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Guttman O, Baranovski BM, Schuster R, Kaner Z, Freixo-Lima GS, Bahar N, et al. (February 2015).
2145: 1876: 1770: 217: 5862: 5625: 5130: 4829: 4767: 4712:: THE S VARIANT OF HUMAN ALPHA1-ANTITRYPSIN, STRUCTURE AND IMPLICATIONS FOR FUNCTION AND METABOLISM 4697:: THE S VARIANT OF HUMAN ALPHA1-ANTITRYPSIN, STRUCTURE AND IMPLICATIONS FOR FUNCTION AND METABOLISM 4682:: THE S VARIANT OF HUMAN ALPHA1-ANTITRYPSIN, STRUCTURE AND IMPLICATIONS FOR FUNCTION AND METABOLISM 4201:"Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury" 3084:
Taggart C, Cervantes-Laurean D, Kim G, McElvaney NG, Wehr N, Moss J, et al. (September 2000).
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Bianchera A, Alomari E, Michielon A, Bazzoli G, Ronda N, Pighini G, et al. (December 2022).
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Miyamoto Y, Akaike T, Maeda H (March 2000). "S-nitrosylated human alpha(1)-protease inhibitor".
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alpha-1 antitrypsin is not yet available for use as a medication but is under development.
2396: 1966: 1902: 1661: 1640:. The three N-linked glycosylations sites are mainly equipped with so-called diantennary N- 1429: 2340: 8: 6115: 5696: 5630: 5588: 5468: 5408: 5154: 5144: 5134: 4918: 4819: 4797: 4792: 4777: 4772: 4445: 2612: 2331: 2034:. Some mutant forms fail to fold properly and are, thus, targeted for destruction in the 1941: 1827: 1774: 1743: 1477: 1410: 4014: 2498: 2475: 6042: 5832: 5640: 5494: 5386: 4424: 4388: 4340: 4315: 4129: 4034: 3960: 3907: 3890: 3866: 3839: 3815: 3790: 3766: 3741: 3061: 3036: 2976: 2892: 2867: 2843: 2818: 2794: 2769: 1888: 262: 4968: 4268: 4243: 4225: 4200: 4088: 3972:
Crystal RG (December 1989). "The alpha 1-antitrypsin gene and its deficiency states".
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Mackiewicz A, Kushner I, Baumann H, eds. (1993). "Acute phase response: an overview".
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encoding the protein can cause misfolding and impaired secretion, which can lead to
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Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology
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Acute phase proteins: molecular biology, biochemistry, and clinical applications
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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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patients. Consequently, carbamylated A1AT is currently being developed as an
1910: 1614: 1552: 701: 4316:"Molecular mousetraps, alpha1-antitrypsin deficiency and the serpinopathies" 3718: 3052: 2660:
ENSG00000277377 GRCh38: Ensembl release 89: ENSG00000197249, ENSG00000277377
1746:, a further elevation is required to "limit" the damage caused by activated 643: 521: 221:, A1A, A1AT, AAT, PI, PI1, PRO2275, alpha1AT, serpin family A member 1, nNIF 6120: 5976: 5959: 5910: 5866: 5747: 5150: 5094: 4943: 4913: 4787: 4782: 4384: 4349: 4306: 4277: 4234: 4191: 4162: 4125: 4096: 4059: 3875: 3824: 3775: 3726: 3182: 3111: 3102: 3085: 3070: 3021: 2972: 2964: 2901: 2852: 2803: 2754: 2581: 2205: 2095:
Other rarer forms have been described; in all, there are over 80 variants.
1996: 1948: 1906: 1872: 1452:. However, defective A1AT may accumulate in the liver, potentially causing 1406: 500: 279: 4067: 4030: 3993: 3956: 3916: 3146: 1299: 1294: 6135: 6037: 6028: 5981: 5971: 5906: 5824: 5809: 5761: 5712: 5701: 5680: 5665: 5509: 5499: 5391: 5342: 5279: 5260: 5220: 5209: 5177: 5017: 2834: 2733:
Gettins PG (December 2002). "Serpin structure, mechanism, and function".
2634: 2190: 1782: 1543:. On electrophoresis, the protein component of the blood is separated by 1437: 1054: 1050: 1031: 3791:"Feasibility of Aerosolized Alpha-1 Antitrypsin as a Therapeutic Option" 2360: 6080: 6032: 5954: 5949: 5939: 5901: 5877: 5557: 5440: 4183: 4154: 3948: 2550: 2371: 2035: 2003: 1918: 1880: 1836: 1778: 1649: 1624:-antitrypsin have been described in various populations. North-Western 1457: 399: 296: 246: 4607:: NON-COVALENT COMPLEX BETWEEN ALPHA-1-PI-PITTSBURGH AND S195A TRYPSIN 4375: 4358: 3346:"Health product highlights 2021: Annexes of products approved in 2021" 2883: 2817:
Winkler IG, Hendy J, Coughlin P, Horvath A, Lévesque JP (April 2005).
2785: 1664:. Also, the fucosylated triantennary N-glycans were shown to have the 1524:
and elsewhere. In older biomedical literature it was sometimes called
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: 41: 6110: 5858: 5795: 5778: 5675: 5445: 5432: 5304: 5284: 5012: 4906: 4901: 4259: 4216: 4022: 2746: 2139: 2084: 2073: 1955: 1925: 1887:). One person with this mutation has been reported to have died of a 1835:. Evidence has shown that cigarette smoke can result in oxidation of 1832: 1684: 1653: 1637: 1517: 1453: 901: 466: 453: 365: 352: 254: 5055: 3083: 6125: 6052: 6023: 5896: 5891: 5655: 5326: 5294: 5097: 4958: 4953: 4938: 4933: 4928: 4896: 4891: 4886: 4881: 4871: 4866: 4856: 4844: 4839: 4834: 4824: 2922:. U.S. National Library of Medicine, National Insittues of Health. 2351: 2167: 2031: 1884: 1786: 1751: 1739: 1711: 1688: 1680: 1572: 1521: 1513: 1386: 1283: 4103: 2443: 5596: 5423: 5366: 5299: 4592:: CRYSTAL STRUCTURE OF A1PI-PITTSBURGH IN THE NATIVE CONFORMATION 4562:: Interactions causing the kinetic trap in serpin protein folding 4287:
Clinica Chimica Acta; International Journal of Clinical Chemistry
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This article incorporates text from this source, which is in the
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nomenclature) is found to be covalently linked to a free single
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Lisowska-Myjak B (February 2005). "AAT as a diagnostic tool".
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endoplasmic reticulum-Golgi intermediate compartment membrane
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in these areas can lead to non-functional proteins that can
5032: 4986: 4622:: INTACT RECOMBINED ALPHA1-ANTITRYPSIN MUTANT PHE 51 TO LEU 3739: 2950: 2816: 1992: 1844: 1679:
particular protein-cell recognition properties. The single
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Brantly ML, Lascano JE, Shahmohammadi A (November 2018).
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endoplasmic reticulum to Golgi vesicle-mediated transport
3195: 2046:. The serum levels of some of the common genotypes are: 1954:
Different analytical methods are used to determine A1AT
1714:, it is considered the most prominent serpin; the terms 490: 3400:"Respreeza - Summary of Product Characteristics (SmPC)" 3159: 3124: 2920:
ClinVar Genomic variation as it relates to human health
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Alpha-1 antitrypsin concentrates are prepared from the
2112: 1970: 1528:(STI, dated terminology), because its capability as a 1496:
versions are also available but are currently used in
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online database for peptidases and their inhibitors:
4169: 2916:"NM_001127701.1(SERPINA1):c.1096G>A (p.Glu366Lys)" 655: 4045: 2091:
mutation at position 264 (288 in pre-processed form)
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mutation at position 342 (366 in pre-processed form)
4074: 3374:"Regulatory Decision Summary for Pronextica Liquid" 4517:: CRYSTAL STRUCTURE OF CLEAVED ANTITRYPSIN POLYMER 3891:"Hereditary variants of serum alpha-1-antitrypsin" 3704: 2676: 2674: 2672: 2655: 2653: 2651: 1898:-positive globules within periportal hepatocytes. 1424:), neutrophil elastase can excessively break down 4356: 3657: 3655: 3653: 3651: 3649: 3647: 2866:Richler R, Forssmann W, Henschler R (June 2017). 2024:and "MM" is the banding pattern of that patient. 437: 336: 6172: 4532:: CRYSTAL STRUCTURE OF A SERPIN:PROTEASE COMPLEX 2946: 2944: 1965:is imprecise, the A1AT phenotype is analysed by 3888: 2669: 2648: 2339: 1571:). The non-albumin proteins are referred to as 1444:, in adults. Normally, A1AT is produced in the 4284: 3644: 3575: 3573: 3034: 2681:GRCm38: Ensembl release 89: ENSMUSG00000071177 5728: 5573: 5071: 4738: 4467: 4241: 3882: 3427: 3425: 2941: 2619:variants of A1AT leading to disease in 1965. 1420:contains inadequate or defective A1AT (as in 857:negative regulation of endopeptidase activity 4357:Rudnick DA, Perlmutter DH (September 2005). 4106:The American Journal of the Medical Sciences 3538: 3536: 3189: 3153: 2993: 2171: 1350:superfamily. It is encoded in humans by the 735:serine-type endopeptidase inhibitor activity 3570: 2726: 2298:In general: ℞ (Prescription only) 2006:results, the IEF results are notated as in 5742: 5735: 5721: 5580: 5566: 5078: 5064: 4745: 4731: 4474: 4460: 4198: 3612: 3610: 3501: 3499: 3464: 3462: 3422: 3028: 2497: 2474: 2111: 1532:was a salient feature of its early study. 761:COPII-coated ER to Golgi transport vesicle 6048:Tetragalacturonic acid hydroxymethylester 5546:disorders of globin and globulin proteins 5046:disorders of globin and globulin proteins 4374: 4339: 4267: 4224: 3934: 3906: 3865: 3855: 3814: 3765: 3533: 3101: 3060: 3011: 2891: 2842: 2793: 2395: 832:negative regulation of peptidase activity 5587: 3889:Axelsson U, Laurell CB (November 1965). 1804: 1800: 811:collagen-containing extracellular matrix 801:intracellular membrane-bounded organelle 3971: 3607: 3496: 3459: 2732: 2470: 2359: 2062:PiSZ: 40% of normal serum level of A1AT 2059:PiMZ: 60% of normal serum level of A1AT 2056:PiSS: 60% of normal serum level of A1AT 2053:PiMS: 80% of normal serum level of A1AT 872:post-translational protein modification 27:Mammalian protein found in Homo sapiens 14: 6173: 4242:Lomas DA, Mahadeva R (December 2002). 3788: 3746:Chronic Obstructive Pulmonary Diseases 3558:from the original on 28 September 2020 3324:Therapeutic Goods Administration (TGA) 3294:Therapeutic Goods Administration (TGA) 3264:Therapeutic Goods Administration (TGA) 2936:Interpretation: Pathogenic risk factor 2774:Clinical & Experimental Immunology 2615:first investigated the possibility of 2488: 2290: 2265: 1520:, is a digestive enzyme active in the 5716: 5561: 5085: 5059: 4726: 4652:: CLEAVED ALPHA-1-ANTITRYPSIN POLYMER 4455: 4313: 4248:The Journal of Clinical Investigation 4205:The Journal of Clinical Investigation 3230:"Alpha-1-Proteinase Inhibitor, Human" 3035:DeMeo DL, Silverman EK (March 2004). 2994:Lapidot T, Petit I (September 2012). 2436: 2415: 2278: 2162: 2144: 1442:chronic obstructive pulmonary disease 442: 403: 398: 341: 300: 295: 4813:Protein Z-related protease inhibitor 2926:from the original on 13 October 2022 2823:The Journal of Experimental Medicine 2442: 2209: 2038:, whereas others have a tendency to 1539:refers to the protein's behavior on 3676:from the original on 3 October 2020 3595:from the original on 3 October 2020 3447:from the original on 10 August 2020 3410:from the original on 3 October 2020 3240:from the original on 3 October 2020 3127:The New England Journal of Medicine 3090:The Journal of Biological Chemistry 1474:an exogenous one used as medication 24: 3927: 3895:American Journal of Human Genetics 3484:from the original on 4 August 2020 3300:from the original on 10 April 2023 3270:from the original on 10 April 2023 2030:levels in the blood depend on the 1894:A liver biopsy will show abundant 1819:Disorders of this protein include 1731:) are often used interchangeably. 25: 6212: 5923:(II, VII, IX, X, protein C and S) 4808:Plasminogen activator inhibitor-2 4803:Plasminogen activator inhibitor-1 4401: 3632:from the original on 1 April 2020 3521:from the original on 1 April 2020 2236: 2130:Aralast, Zemaira, Glassia, others 1683:residue of A1AT in position 256 ( 6158: 4702: 4687: 4672: 4657: 4642: 4627: 4612: 4597: 4582: 4567: 4552: 4537: 4522: 4507: 4492: 4172:Biochemical Society Transactions 4143:Biochemical Society Transactions 4118:10.1097/00000441-200101000-00006 3686: 2528: 2522: 1656:amino acid nomenclature). These 1620:Over 100 different variants of α 1470:an endogenous protease inhibitor 671: 664: 426: 419: 413: 390: 325: 318: 312: 287: 40: 5966:thrombopoietin receptor agonist 5921:Prothrombin complex concentrate 4502:: UNCLEAVED ALPHA-1-ANTITRYPSIN 4481: 4199:Perlmutter DH (December 2002). 3831: 3782: 3733: 3392: 3378:Drug and Health Products Portal 3366: 3338: 3312: 3282: 3252: 3222: 3118: 3077: 2987: 2098: 1797:(infantile hepatic cirrhosis). 1503: 6058:Hemostatic Powder Spray TC-325 5525:Extracellular matrix protein 1 2908: 2859: 2810: 2761: 2708: 2690: 2540: 2534: 2516: 2249: 2067:alpha-1 antitrypsin deficiency 1982:alpha-1 antitrypsin deficiency 1821:alpha-1 antitrypsin deficiency 1815:Alpha-1 antitrypsin deficiency 1613:is located on the long arm of 1422:alpha-1 antitrypsin deficiency 1385:) because it inhibits various 682:More reference expression data 644:More reference expression data 13: 1: 4089:10.1016/S0167-4838(99)00264-2 3857:10.3390/pharmaceutics14122754 3807:10.15326/jcopdf.7.3.2019.0179 3758:10.15326/jcopdf.6.1.2017.0185 3013:10.1016/s0301-472x(02)00883-4 2641: 1905:(RA) have been found to make 411: 310: 6181:Genes on human chromosome 14 5256:Sex hormone-binding globulin 4332:10.7861/clinmedicine.5-3-249 4048:Journal of Molecular Biology 3986:10.1016/0168-9525(89)90200-X 3624:Food and Drug Administration 3587:Food and Drug Administration 3550:Food and Drug Administration 3513:Food and Drug Administration 3476:Food and Drug Administration 3439:Food and Drug Administration 3200:. CRC Press. pp. 3–19. 2637:is a related alpha 1 protein 2586:Food and Drug Administration 2322:Alpha-1-proteinase inhibitor 2173:Alpha 1 proteinase inhibitor 1773:, A1AT has a characteristic 1631: 1595:alpha-1 proteinase inhibitor 806:ficolin-1-rich granule lumen 776:platelet alpha granule lumen 715:peptidase inhibitor activity 7: 5671:Haptoglobin-related protein 4752: 3139:10.1056/NEJM198309223091203 2622: 1931: 1698: 1604: 756:endoplasmic reticulum lumen 10: 6217: 6201:Serine protease inhibitors 5853:Valoctocogene roxaparvovec 5023:Thyroxine-binding globulin 4444:gene details page in the 4299:10.1016/j.cccn.2004.03.012 3175:10.1016/j.jaut.2017.02.008 2606: 2506:Chemical and physical data 1854:An extremely rare form of 1812: 1771:serine protease inhibitors 1675:, which could confer this 1260:Chr 12: 103.73 – 103.74 Mb 6070: 6014:Absorbable gelatin sponge 6006: 5930: 5808: 5777: 5770: 5755: 5689: 5618: 5595: 5537: 5520:Vitamin D-binding protein 5487: 5459: 5431: 5422: 5379: 5335: 5317: 5270: 5243: 5234: 5199: 5164: 5114: 5105: 5093: 5041: 4995: 4967: 4760: 4487: 3668:European Medicines Agency 2716:"Mouse PubMed Reference:" 2698:"Human PubMed Reference:" 2566: 2549: 2510: 2505: 2486: 2454: 2426: 2406: 2386: 2370: 2350: 2330: 2310: 2305: 2226: 2221: 2196: 2182: 2152: 2134: 2124: 2119: 2110: 1500:more than as medication. 1464:Paneth cells of the gut. 1428:, leading to the loss of 1298: 1293: 1289: 1282: 1266: 1247: 1224: 1220: 1183: 1159: 1152: 1137: 1133: 1096: 1072: 1065: 1048: 1044: 1029: 1025: 1016: 1003: 999: 980: 976: 967: 954: 950: 935: 931: 922: 907: 900: 896: 880: 730:identical protein binding 700: 696: 679: 663: 654: 641: 590: 581: 528: 519: 489: 481: 477: 460: 447: 410: 389: 380: 376: 359: 346: 309: 286: 277: 273: 228: 225: 215: 208: 203: 84: 79: 62: 57: 52: 48: 39: 34: 5863:Fidanacogene elaparvovec 5626:Alpha 1-antichymotrypsin 5131:Alpha 1-antichymotrypsin 4768:Alpha 1-antichymotrypsin 2629:Alpha 1-antichymotrypsin 2584:of blood donors. The US 2042:, being retained in the 1987:As every person has two 1938:reference range in blood 1754:, which breaks down the 1734:Most serpins inactivate 1253:Chr 14: 94.38 – 94.39 Mb 867:neutrophil degranulation 6097:Aminomethylbenzoic acid 5414:7S seed storage protein 5191:Retinol binding protein 3789:Usmani OS (July 2020). 3719:10.1378/chest.117.3.875 3163:Journal of Autoimmunity 3053:10.1136/thx.2003.006502 1748:neutrophil granulocytes 1668:as part of a so-called 1648:can be attached to the 1555:, the second being the 1541:protein electrophoresis 1526:serum trypsin inhibitor 564:upper lobe of left lung 4969:Cross class inhibitory 4060:10.1006/jmbi.1997.1458 3795:Chronic Obstr Pulm Dis 3103:10.1074/jbc.M004850200 2965:10.1002/pmic.200500751 1976:Normal A1AT is termed 1879:), due to a mutation ( 1810: 1793:and accumulate in the 1484:and is sold under the 1480:form is purified from 1361:, it is also known as 837:platelet degranulation 6191:Enzymes used as drugs 5829:Damoctocog alfa pegol 5636:Alpha 2-macroglobulin 5217:alpha-2-Macroglobulin 5003:Heat shock protein 47 3672:. 17 September 2018. 3591:. 21 September 2017. 2872:Transfus Med Hemother 2065:PiZZ: 10-15% (severe 2044:endoplasmic reticulum 1825:autosomal co-dominant 1808: 1801:Clinical significance 1593:Another name used is 1516:. Trypsin, a type of 1367:–proteinase inhibitor 847:COPII vesicle coating 786:endoplasmic reticulum 766:extracellular exosome 444:12 E|12 52.98 cM 405:Chromosome 12 (mouse) 303:Chromosome 14 (human) 6186:Acute-phase proteins 5589:Acute-phase proteins 5474:Bovine serum albumin 5290:Beta-2 microglobulin 4577:: ALPHA1-ANTITRYPSIN 4438:genome location and 4418:1 April 2008 at the 2835:10.1084/jem.20042299 1967:isoelectric focusing 1913:form of A1AT in the 1903:rheumatoid arthritis 1744:acute phase reaction 1662:isoelectric focusing 1547:. There are several 1450:systemic circulation 852:acute-phase response 781:extracellular region 598:hepatobiliary system 267:SERPINA1 - orthologs 80:List of PDB id codes 53:Available structures 5631:Alpha 1-antitrypsin 5469:Human serum albumin 5409:11S globulin family 5155:Heparin cofactor II 5145:Alpha 2-antiplasmin 5135:Alpha 1-antitrypsin 4798:Protein C inhibitor 4793:Heparin cofactor II 4778:Alpha 2-antiplasmin 4773:Alpha 1-antitrypsin 4446:UCSC Genome Browser 4427:Alpha-1-antitrypsin 4015:1982Natur.298..329C 3406:. 10 October 2018. 2274:(Prescription only) 2245:(Prescription only) 2107: 2105:Alpha-1 antitrypsin 2083:PiS is caused by a 2072:PiZ is caused by a 2050:PiMM: 100% (normal) 2028:Alpha-1 antitrypsin 1833:pulmonary emphysema 1828:hereditary disorder 1775:secondary structure 1738:by binding to them 1486:nonproprietary name 1456:in both adults and 1411:neutrophil elastase 1312:Alpha-1 antitrypsin 796:extracellular space 630:islet of Langerhans 540:islet of Langerhans 532:right lobe of liver 6043:Oxidized cellulose 5833:Efanesoctocog alfa 5641:C-reactive protein 5495:C-reactive protein 5387:Beta-lactoglobulin 4184:10.1042/BST0300093 4155:10.1042/BST0300089 3974:Trends in Genetics 3949:10.1007/BF01115992 3937:Bioscience Reports 3380:. 14 November 2023 2103: 2022:protease inhibitor 1942:acute inflammation 1889:bleeding diathesis 1871:, functions as an 1811: 1721:protease inhibitor 1551:, the first being 1438:respiratory issues 1436:. This results in 1409:cells, especially 1359:protease inhibitor 1006:ENSMUSG00000071177 820:Biological process 744:Cellular component 708:Molecular function 6146: 6145: 6116:Alfa1 antitrypsin 6087:Aminocaproic acid 6072:Antifibrinolytics 6066: 6065: 6002: 6001: 5837:Efmoroctocog alfa 5787:Phytomenadione (K 5710: 5709: 5555: 5554: 5533: 5532: 5504:Alpha-lactalbumin 5404:Alpha-lactalbumin 5375: 5374: 5347:Fetal fibronectin 5313: 5312: 5230: 5229: 5087:Globular proteins 5053: 5052: 4720: 4719: 4376:10.1002/hep.20815 4320:Clinical Medicine 4314:Lomas DA (2005). 2884:10.1159/000477262 2786:10.1111/cei.12476 2578: 2577: 2456:CompTox Dashboard 2294: 2282: 2269: 2252: 2240: 2165: 1756:connective tissue 1750:and their enzyme 1530:trypsin inhibitor 1482:human donor blood 1393:). As a type of 1346:belonging to the 1309: 1308: 1305: 1304: 1278: 1277: 1243: 1242: 1214: 1213: 1148: 1147: 1127: 1126: 1061: 1060: 1038: 1037: 1012: 1011: 993: 992: 963: 962: 944: 943: 892: 891: 862:blood coagulation 692: 691: 688: 687: 650: 649: 637: 636: 575: 574: 473: 472: 372: 371: 199: 198: 195: 194: 63:Ortholog search: 16:(Redirected from 6208: 6163: 6162: 6161: 6154: 6019:Calcium alginate 5775: 5774: 5768: 5767: 5757:Antihemorrhagics 5744:Antihemorrhagics 5737: 5730: 5723: 5714: 5713: 5582: 5575: 5568: 5559: 5558: 5429: 5428: 5273: 5249: 5246:carrier proteins 5241: 5240: 5202: 5170: 5167:carrier proteins 5120: 5112: 5111: 5103: 5102: 5080: 5073: 5066: 5057: 5056: 4747: 4740: 4733: 4724: 4723: 4706: 4691: 4676: 4661: 4646: 4631: 4616: 4601: 4586: 4571: 4556: 4541: 4526: 4511: 4496: 4476: 4469: 4462: 4453: 4452: 4396: 4378: 4353: 4343: 4310: 4281: 4271: 4260:10.1172/JCI16782 4238: 4228: 4217:10.1172/JCI16787 4195: 4166: 4137: 4100: 4071: 4042: 4023:10.1038/298329a0 4009:(5872): 329–34. 3997: 3968: 3921: 3920: 3910: 3886: 3880: 3879: 3869: 3859: 3835: 3829: 3828: 3818: 3786: 3780: 3779: 3769: 3737: 3731: 3730: 3702: 3696: 3690: 3689: 3685: 3683: 3681: 3663:"Respreeza EPAR" 3659: 3642: 3641: 3639: 3637: 3628:. 22 July 2017. 3614: 3605: 3604: 3602: 3600: 3577: 3568: 3567: 3565: 3563: 3554:. 5 March 2018. 3540: 3531: 3530: 3528: 3526: 3517:. 22 July 2017. 3503: 3494: 3493: 3491: 3489: 3480:. 22 July 2017. 3466: 3457: 3456: 3454: 3452: 3443:. 22 July 2017. 3429: 3420: 3419: 3417: 3415: 3396: 3390: 3389: 3387: 3385: 3370: 3364: 3363: 3361: 3359: 3342: 3336: 3335: 3333: 3331: 3316: 3310: 3309: 3307: 3305: 3296:. 21 June 2022. 3286: 3280: 3279: 3277: 3275: 3266:. 21 June 2022. 3256: 3250: 3249: 3247: 3245: 3226: 3220: 3219: 3193: 3187: 3186: 3157: 3151: 3150: 3122: 3116: 3115: 3105: 3096:(35): 27258–65. 3081: 3075: 3074: 3064: 3032: 3026: 3025: 3015: 2991: 2985: 2984: 2948: 2939: 2938: 2933: 2931: 2912: 2906: 2905: 2895: 2863: 2857: 2856: 2846: 2814: 2808: 2807: 2797: 2765: 2759: 2758: 2747:10.1021/cr010170 2741:(12): 4751–804. 2735:Chemical Reviews 2730: 2724: 2723: 2712: 2706: 2705: 2694: 2688: 2678: 2667: 2657: 2574: 2573: 2561: 2559: 2542: 2536: 2530: 2524: 2518: 2501: 2490: 2479: 2478: 2464: 2462: 2446: 2440: 2419: 2399: 2363: 2343: 2292: 2289: 2280: 2277: 2267: 2264: 2260: 2251: 2248: 2238: 2235: 2213: 2175: 2164: 2161: 2148: 2115: 2108: 2106: 2102: 1766:responsiveness. 1693:disulfide bridge 1545:electric current 1498:medical research 1395:enzyme inhibitor 1291: 1290: 1262: 1255: 1238: 1218: 1217: 1209: 1157: 1156: 1153:RefSeq (protein) 1143: 1131: 1130: 1122: 1070: 1069: 1042: 1041: 1023: 1022: 997: 996: 974: 973: 948: 947: 929: 928: 898: 897: 720:protease binding 698: 697: 684: 675: 668: 661: 660: 646: 586: 584:Top expressed in 579: 578: 524: 522:Top expressed in 517: 516: 496: 495: 479: 478: 469: 456: 445: 430: 423: 417: 406: 394: 378: 377: 368: 355: 344: 329: 322: 316: 305: 291: 275: 274: 269: 220: 213: 190: 77: 76: 71: 50: 49: 44: 32: 31: 21: 6216: 6215: 6211: 6210: 6209: 6207: 6206: 6205: 6171: 6170: 6169: 6159: 6157: 6149: 6147: 6142: 6092:Tranexamic acid 6062: 5998: 5932: 5926: 5849:Turoctocog alfa 5845:Susoctocog alfa 5841:Moroctocog alfa 5811: 5804: 5799: 5790: 5759: 5751: 5741: 5711: 5706: 5685: 5614: 5591: 5586: 5556: 5551: 5550: 5529: 5483: 5455: 5418: 5380:Other globulins 5371: 5331: 5327:Immunoglobulins 5309: 5271: 5266: 5263: 5244: 5226: 5200: 5195: 5165: 5160: 5115: 5107:Alpha globulins 5089: 5084: 5054: 5049: 5037: 5028:Angiotensinogen 4991: 4963: 4756: 4751: 4721: 4716: 4713: 4707: 4698: 4692: 4683: 4677: 4668: 4662: 4653: 4647: 4638: 4632: 4623: 4617: 4608: 4602: 4593: 4587: 4578: 4572: 4563: 4557: 4548: 4542: 4533: 4527: 4518: 4512: 4503: 4497: 4483: 4480: 4420:Wayback Machine 4404: 4399: 4254:(11): 1585–90. 4211:(11): 1579–83. 3930: 3928:Further reading 3925: 3924: 3887: 3883: 3836: 3832: 3787: 3783: 3738: 3734: 3703: 3699: 3687: 3679: 3677: 3661: 3660: 3645: 3635: 3633: 3616: 3615: 3608: 3598: 3596: 3579: 3578: 3571: 3561: 3559: 3542: 3541: 3534: 3524: 3522: 3505: 3504: 3497: 3487: 3485: 3468: 3467: 3460: 3450: 3448: 3431: 3430: 3423: 3413: 3411: 3398: 3397: 3393: 3383: 3381: 3372: 3371: 3367: 3357: 3355: 3354:. 3 August 2022 3344: 3343: 3339: 3329: 3327: 3318: 3317: 3313: 3303: 3301: 3288: 3287: 3283: 3273: 3271: 3258: 3257: 3253: 3243: 3241: 3228: 3227: 3223: 3208: 3194: 3190: 3158: 3154: 3123: 3119: 3082: 3078: 3033: 3029: 2992: 2988: 2959:(11): 3369–80. 2949: 2942: 2929: 2927: 2914: 2913: 2909: 2864: 2860: 2815: 2811: 2766: 2762: 2731: 2727: 2714: 2713: 2709: 2696: 2695: 2691: 2679: 2670: 2658: 2649: 2644: 2625: 2609: 2569: 2567: 2557: 2555: 2545: 2539: 2533: 2527: 2521: 2482: 2458: 2450: 2422: 2402: 2382: 2366: 2346: 2326: 2323: 2318: 2317: 2301: 2258: 2217: 2185: 2178: 2101: 2018: 2011: 1963:electrophoresis 1934: 1870: 1866: 1859: 1849:liver cirrhosis 1842: 1817: 1803: 1729: 1701: 1634: 1623: 1607: 1600: 1569:immunoglobulins 1563:and the fourth 1506: 1491: 1448:and enters the 1379:-antiproteinase 1378: 1366: 1331: 1319: 1300:View/Edit Mouse 1295:View/Edit Human 1258: 1251: 1248:Location (UCSC) 1234: 1230: 1226: 1205: 1201: 1197: 1193: 1189: 1185: 1179: 1175: 1171: 1167: 1163: 1139: 1118: 1114: 1110: 1106: 1102: 1098: 1092: 1088: 1084: 1080: 1076: 1053: 987:ENSG00000277377 985: 983:ENSG00000197249 876: 815: 751:Golgi apparatus 739: 725:protein binding 680: 670: 669: 642: 633: 628: 624: 620: 616: 612: 608: 604: 600: 596: 594:proximal tubule 582: 571: 566: 562: 558: 554: 550: 546: 542: 538: 534: 520: 464: 451: 443: 433: 432: 431: 424: 404: 381:Gene location ( 363: 350: 342: 332: 331: 330: 323: 301: 278:Gene location ( 229: 216: 209: 86: 64: 28: 23: 22: 15: 12: 11: 5: 6214: 6204: 6203: 6198: 6193: 6188: 6183: 6168: 6167: 6144: 6143: 6141: 6140: 6139: 6138: 6130: 6129: 6128: 6123: 6118: 6113: 6101: 6100: 6099: 6094: 6089: 6076: 6074: 6068: 6067: 6064: 6063: 6061: 6060: 6055: 6050: 6045: 6040: 6035: 6026: 6021: 6016: 6010: 6008: 6004: 6003: 6000: 5999: 5997: 5996: 5995: 5994: 5989: 5984: 5979: 5974: 5962: 5957: 5952: 5947: 5942: 5936: 5934: 5928: 5927: 5925: 5924: 5914: 5913: 5904: 5899: 5894: 5885: 5884: 5871: 5870: 5856: 5822: 5816: 5814: 5806: 5805: 5803: 5802: 5797: 5793: 5788: 5783: 5781: 5772: 5765: 5753: 5752: 5740: 5739: 5732: 5725: 5717: 5708: 5707: 5705: 5704: 5699: 5693: 5691: 5687: 5686: 5684: 5683: 5678: 5673: 5668: 5663: 5658: 5653: 5648: 5643: 5638: 5633: 5628: 5622: 5620: 5619:Other positive 5616: 5615: 5613: 5612: 5607: 5601: 5599: 5593: 5592: 5585: 5584: 5577: 5570: 5562: 5553: 5552: 5549: 5548: 5539: 5538: 5535: 5534: 5531: 5530: 5528: 5527: 5522: 5517: 5512: 5507: 5497: 5491: 5489: 5485: 5484: 5482: 5481: 5476: 5471: 5465: 5463: 5457: 5456: 5454: 5453: 5448: 5443: 5437: 5435: 5426: 5420: 5419: 5417: 5416: 5411: 5406: 5401: 5396: 5395: 5394: 5383: 5381: 5377: 5376: 5373: 5372: 5370: 5369: 5364: 5362:Transcobalamin 5359: 5350: 5339: 5337: 5333: 5332: 5330: 5329: 5323: 5321: 5319:Gamma globulin 5315: 5314: 5311: 5310: 5308: 5307: 5302: 5297: 5292: 5287: 5282: 5276: 5274: 5268: 5267: 5265: 5264: 5258: 5252: 5250: 5238: 5236:Beta globulins 5232: 5231: 5228: 5227: 5225: 5224: 5213: 5205: 5203: 5197: 5196: 5194: 5193: 5188: 5181: 5173: 5171: 5162: 5161: 5159: 5158: 5148: 5138: 5123: 5121: 5109: 5100: 5091: 5090: 5083: 5082: 5075: 5068: 5060: 5051: 5050: 5042: 5039: 5038: 5036: 5035: 5030: 5025: 5020: 5015: 5010: 5005: 4999: 4997: 4993: 4992: 4990: 4989: 4984: 4979: 4973: 4971: 4965: 4964: 4962: 4961: 4956: 4951: 4946: 4941: 4936: 4931: 4926: 4921: 4916: 4910: 4909: 4904: 4899: 4894: 4889: 4884: 4879: 4874: 4869: 4864: 4859: 4853: 4852: 4847: 4842: 4837: 4832: 4827: 4822: 4816: 4815: 4810: 4805: 4800: 4795: 4790: 4785: 4780: 4775: 4770: 4764: 4762: 4758: 4757: 4750: 4749: 4742: 4735: 4727: 4718: 4717: 4715: 4714: 4708: 4701: 4699: 4693: 4686: 4684: 4678: 4671: 4669: 4663: 4656: 4654: 4648: 4641: 4639: 4633: 4626: 4624: 4618: 4611: 4609: 4603: 4596: 4594: 4588: 4581: 4579: 4573: 4566: 4564: 4558: 4551: 4549: 4543: 4536: 4534: 4528: 4521: 4519: 4513: 4506: 4504: 4498: 4491: 4488: 4485: 4484: 4479: 4478: 4471: 4464: 4456: 4450: 4449: 4430: 4422: 4403: 4402:External links 4400: 4398: 4397: 4354: 4311: 4282: 4239: 4196: 4167: 4138: 4101: 4072: 4043: 3998: 3969: 3931: 3929: 3926: 3923: 3922: 3881: 3830: 3781: 3752:(1): 100–114. 3732: 3697: 3643: 3606: 3569: 3532: 3495: 3458: 3421: 3391: 3365: 3337: 3326:. 21 June 2022 3311: 3281: 3251: 3236:. 4 May 2020. 3221: 3206: 3188: 3152: 3117: 3076: 3027: 3006:(9): 973–981. 2986: 2940: 2907: 2878:(3): 151–164. 2858: 2829:(7): 1077–88. 2809: 2780:(2): 161–172. 2760: 2725: 2707: 2689: 2668: 2646: 2645: 2643: 2640: 2639: 2638: 2632: 2624: 2621: 2608: 2605: 2576: 2575: 2564: 2563: 2553: 2547: 2546: 2543: 2537: 2531: 2525: 2519: 2514: 2508: 2507: 2503: 2502: 2492: 2484: 2483: 2481: 2480: 2467: 2465: 2452: 2451: 2449: 2448: 2432: 2430: 2424: 2423: 2421: 2420: 2412: 2410: 2404: 2403: 2401: 2400: 2392: 2390: 2384: 2383: 2381: 2380: 2376: 2374: 2368: 2367: 2365: 2364: 2356: 2354: 2348: 2347: 2345: 2344: 2336: 2334: 2328: 2327: 2325: 2324: 2321: 2313: 2312: 2311: 2308: 2307: 2303: 2302: 2300: 2299: 2296: 2287: 2275: 2262: 2246: 2232: 2230: 2224: 2223: 2219: 2218: 2216: 2215: 2202: 2200: 2194: 2193: 2188: 2186:administration 2180: 2179: 2177: 2176: 2158: 2156: 2150: 2149: 2142: 2132: 2131: 2128: 2122: 2121: 2117: 2116: 2100: 2097: 2093: 2092: 2081: 2070: 2063: 2060: 2057: 2054: 2051: 2016: 2009: 1933: 1930: 1915:synovial fluid 1907:autoantibodies 1901:Patients with 1868: 1864: 1857: 1840: 1813:Main article: 1802: 1799: 1727: 1716:α1-antitrypsin 1700: 1697: 1670:Sialyl Lewis x 1633: 1630: 1621: 1606: 1603: 1598: 1588:alpha-globulin 1505: 1502: 1489: 1478:pharmaceutical 1397:, it protects 1376: 1364: 1329: 1317: 1307: 1306: 1303: 1302: 1297: 1287: 1286: 1280: 1279: 1276: 1275: 1273: 1271: 1264: 1263: 1256: 1249: 1245: 1244: 1241: 1240: 1222: 1221: 1215: 1212: 1211: 1181: 1180: 1154: 1150: 1149: 1146: 1145: 1135: 1134: 1128: 1125: 1124: 1094: 1093: 1067: 1063: 1062: 1059: 1058: 1046: 1045: 1039: 1036: 1035: 1027: 1026: 1020: 1014: 1013: 1010: 1009: 1001: 1000: 994: 991: 990: 978: 977: 971: 965: 964: 961: 960: 952: 951: 945: 942: 941: 933: 932: 926: 920: 919: 914: 909: 905: 904: 894: 893: 890: 889: 878: 877: 875: 874: 869: 864: 859: 854: 849: 844: 839: 834: 829: 823: 821: 817: 816: 814: 813: 808: 803: 798: 793: 791:Golgi membrane 788: 783: 778: 773: 768: 763: 758: 753: 747: 745: 741: 740: 738: 737: 732: 727: 722: 717: 711: 709: 705: 704: 694: 693: 690: 689: 686: 685: 677: 676: 658: 652: 651: 648: 647: 639: 638: 635: 634: 632: 631: 627: 623: 619: 615: 611: 607: 603: 599: 595: 591: 588: 587: 576: 573: 572: 570: 569: 565: 561: 557: 553: 549: 545: 541: 537: 533: 529: 526: 525: 513: 512: 504: 493: 487: 486: 483:RNA expression 475: 474: 471: 470: 462: 458: 457: 449: 446: 441: 435: 434: 425: 418: 412: 408: 407: 402: 396: 395: 387: 386: 374: 373: 370: 369: 361: 357: 356: 348: 345: 340: 334: 333: 324: 317: 311: 307: 306: 299: 293: 292: 284: 283: 271: 270: 227: 223: 222: 214: 206: 205: 201: 200: 197: 196: 193: 192: 82: 81: 73: 72: 61: 55: 54: 46: 45: 37: 36: 26: 9: 6: 4: 3: 2: 6213: 6202: 6199: 6197: 6194: 6192: 6189: 6187: 6184: 6182: 6179: 6178: 6176: 6166: 6156: 6155: 6152: 6137: 6134: 6133: 6131: 6127: 6124: 6122: 6119: 6117: 6114: 6112: 6109: 6108: 6107: 6106: 6102: 6098: 6095: 6093: 6090: 6088: 6085: 6084: 6083: 6082: 6078: 6077: 6075: 6073: 6069: 6059: 6056: 6054: 6051: 6049: 6046: 6044: 6041: 6039: 6036: 6034: 6030: 6027: 6025: 6022: 6020: 6017: 6015: 6012: 6011: 6009: 6005: 5993: 5992:Rafutrombopag 5990: 5988: 5987:Lusutrombopag 5985: 5983: 5980: 5978: 5975: 5973: 5970: 5969: 5968: 5967: 5963: 5961: 5958: 5956: 5953: 5951: 5948: 5946: 5945:Carbazochrome 5943: 5941: 5938: 5937: 5935: 5929: 5922: 5919: 5918:combinations: 5916: 5915: 5912: 5908: 5905: 5903: 5900: 5898: 5895: 5893: 5890: 5887: 5886: 5883: 5879: 5876: 5873: 5872: 5868: 5864: 5860: 5857: 5854: 5850: 5846: 5842: 5838: 5834: 5830: 5826: 5823: 5821: 5818: 5817: 5815: 5813: 5807: 5801: 5794: 5792: 5785: 5784: 5782: 5780: 5776: 5773: 5769: 5766: 5763: 5758: 5754: 5749: 5745: 5738: 5733: 5731: 5726: 5724: 5719: 5718: 5715: 5703: 5700: 5698: 5697:Serum albumin 5695: 5694: 5692: 5688: 5682: 5679: 5677: 5674: 5672: 5669: 5667: 5664: 5662: 5659: 5657: 5654: 5652: 5649: 5647: 5646:Ceruloplasmin 5644: 5642: 5639: 5637: 5634: 5632: 5629: 5627: 5624: 5623: 5621: 5617: 5611: 5608: 5606: 5603: 5602: 5600: 5598: 5594: 5590: 5583: 5578: 5576: 5571: 5569: 5564: 5563: 5560: 5547: 5544: 5541: 5540: 5536: 5526: 5523: 5521: 5518: 5516: 5513: 5511: 5508: 5505: 5501: 5498: 5496: 5493: 5492: 5490: 5486: 5480: 5477: 5475: 5472: 5470: 5467: 5466: 5464: 5462: 5461:Serum albumin 5458: 5452: 5449: 5447: 5444: 5442: 5439: 5438: 5436: 5434: 5430: 5427: 5425: 5421: 5415: 5412: 5410: 5407: 5405: 5402: 5400: 5399:Thyroglobulin 5397: 5393: 5390: 5389: 5388: 5385: 5384: 5382: 5378: 5368: 5365: 5363: 5360: 5358: 5357:Microglobulin 5354: 5353:Macroglobulin 5351: 5348: 5344: 5341: 5340: 5338: 5334: 5328: 5325: 5324: 5322: 5320: 5316: 5306: 5303: 5301: 5298: 5296: 5293: 5291: 5288: 5286: 5283: 5281: 5278: 5277: 5275: 5269: 5262: 5259: 5257: 5254: 5253: 5251: 5247: 5242: 5239: 5237: 5233: 5222: 5218: 5214: 5211: 5207: 5206: 5204: 5198: 5192: 5189: 5186: 5185:Ceruloplasmin 5182: 5179: 5175: 5174: 5172: 5168: 5163: 5156: 5152: 5149: 5146: 5142: 5139: 5136: 5132: 5128: 5125: 5124: 5122: 5118: 5113: 5110: 5108: 5104: 5101: 5099: 5096: 5092: 5088: 5081: 5076: 5074: 5069: 5067: 5062: 5061: 5058: 5048: 5047: 5040: 5034: 5031: 5029: 5026: 5024: 5021: 5019: 5016: 5014: 5011: 5009: 5006: 5004: 5001: 5000: 4998: 4996:noninhibitory 4994: 4988: 4985: 4983: 4980: 4978: 4975: 4974: 4972: 4970: 4966: 4960: 4957: 4955: 4952: 4950: 4947: 4945: 4942: 4940: 4937: 4935: 4932: 4930: 4927: 4925: 4922: 4920: 4917: 4915: 4912: 4911: 4908: 4905: 4903: 4900: 4898: 4895: 4893: 4890: 4888: 4885: 4883: 4880: 4878: 4875: 4873: 4870: 4868: 4865: 4863: 4860: 4858: 4855: 4854: 4851: 4848: 4846: 4843: 4841: 4838: 4836: 4833: 4831: 4828: 4826: 4823: 4821: 4818: 4817: 4814: 4811: 4809: 4806: 4804: 4801: 4799: 4796: 4794: 4791: 4789: 4786: 4784: 4781: 4779: 4776: 4774: 4771: 4769: 4766: 4765: 4763: 4759: 4755: 4748: 4743: 4741: 4736: 4734: 4729: 4728: 4725: 4711: 4705: 4700: 4696: 4690: 4685: 4681: 4675: 4670: 4666: 4660: 4655: 4651: 4645: 4640: 4636: 4630: 4625: 4621: 4615: 4610: 4606: 4600: 4595: 4591: 4585: 4580: 4576: 4570: 4565: 4561: 4555: 4550: 4546: 4540: 4535: 4531: 4525: 4520: 4516: 4510: 4505: 4501: 4495: 4490: 4489: 4486: 4477: 4472: 4470: 4465: 4463: 4458: 4457: 4454: 4447: 4443: 4442: 4437: 4436: 4431: 4429: 4428: 4425:Proteopedia: 4423: 4421: 4417: 4414: 4410: 4406: 4405: 4394: 4390: 4386: 4382: 4377: 4372: 4369:(3): 514–21. 4368: 4364: 4360: 4355: 4351: 4347: 4342: 4337: 4333: 4329: 4326:(3): 249–57. 4325: 4321: 4317: 4312: 4308: 4304: 4300: 4296: 4293:(1–2): 1–13. 4292: 4288: 4283: 4279: 4275: 4270: 4265: 4261: 4257: 4253: 4249: 4245: 4240: 4236: 4232: 4227: 4222: 4218: 4214: 4210: 4206: 4202: 4197: 4193: 4189: 4185: 4181: 4177: 4173: 4168: 4164: 4160: 4156: 4152: 4148: 4144: 4139: 4135: 4131: 4127: 4123: 4119: 4115: 4111: 4107: 4102: 4098: 4094: 4090: 4086: 4083:(1–2): 90–7. 4082: 4078: 4073: 4069: 4065: 4061: 4057: 4054:(3): 419–25. 4053: 4049: 4044: 4040: 4036: 4032: 4028: 4024: 4020: 4016: 4012: 4008: 4004: 3999: 3995: 3991: 3987: 3983: 3980:(12): 411–7. 3979: 3975: 3970: 3966: 3962: 3958: 3954: 3950: 3946: 3943:(2): 129–38. 3942: 3938: 3933: 3932: 3918: 3914: 3909: 3904: 3901:(6): 466–72. 3900: 3896: 3892: 3885: 3877: 3873: 3868: 3863: 3858: 3853: 3849: 3845: 3844:Pharmaceutics 3841: 3834: 3826: 3822: 3817: 3812: 3808: 3804: 3800: 3796: 3792: 3785: 3777: 3773: 3768: 3763: 3759: 3755: 3751: 3747: 3743: 3736: 3728: 3724: 3720: 3716: 3713:(3): 875–80. 3712: 3708: 3701: 3694: 3693:public domain 3675: 3671: 3669: 3664: 3658: 3656: 3654: 3652: 3650: 3648: 3631: 3627: 3625: 3619: 3613: 3611: 3594: 3590: 3588: 3582: 3581:"Prolastin-C" 3576: 3574: 3557: 3553: 3551: 3545: 3539: 3537: 3520: 3516: 3514: 3508: 3502: 3500: 3483: 3479: 3477: 3471: 3465: 3463: 3446: 3442: 3440: 3434: 3428: 3426: 3409: 3405: 3401: 3395: 3379: 3375: 3369: 3353: 3352: 3351:Health Canada 3347: 3341: 3325: 3321: 3315: 3299: 3295: 3291: 3285: 3269: 3265: 3261: 3255: 3239: 3235: 3231: 3225: 3217: 3213: 3209: 3207:1-000-14197-7 3203: 3199: 3192: 3184: 3180: 3176: 3172: 3168: 3164: 3156: 3148: 3144: 3140: 3136: 3133:(12): 694–8. 3132: 3128: 3121: 3113: 3109: 3104: 3099: 3095: 3091: 3087: 3080: 3072: 3068: 3063: 3058: 3054: 3050: 3047:(3): 259–64. 3046: 3042: 3038: 3031: 3023: 3019: 3014: 3009: 3005: 3001: 2997: 2990: 2982: 2978: 2974: 2970: 2966: 2962: 2958: 2954: 2947: 2945: 2937: 2925: 2921: 2917: 2911: 2903: 2899: 2894: 2889: 2885: 2881: 2877: 2873: 2869: 2862: 2854: 2850: 2845: 2840: 2836: 2832: 2828: 2824: 2820: 2813: 2805: 2801: 2796: 2791: 2787: 2783: 2779: 2775: 2771: 2764: 2756: 2752: 2748: 2744: 2740: 2736: 2729: 2721: 2717: 2711: 2703: 2699: 2693: 2686: 2682: 2677: 2675: 2673: 2665: 2661: 2656: 2654: 2652: 2647: 2636: 2633: 2630: 2627: 2626: 2620: 2618: 2614: 2611:Axelsson and 2604: 2602: 2596: 2593: 2589: 2587: 2583: 2572: 2565: 2554: 2552: 2548: 2515: 2513: 2509: 2504: 2500: 2496: 2493: 2491: 2489:ECHA InfoCard 2485: 2477: 2473: 2472:DTXSID2040810 2469: 2468: 2466: 2457: 2453: 2445: 2444:RCSB PDB 2439: 2434: 2433: 2431: 2429: 2425: 2418: 2417:ChEMBL4297879 2414: 2413: 2411: 2409: 2405: 2398: 2394: 2393: 2391: 2389: 2385: 2378: 2377: 2375: 2373: 2369: 2362: 2358: 2357: 2355: 2353: 2349: 2342: 2338: 2337: 2335: 2333: 2329: 2320: 2319: 2316: 2309: 2304: 2297: 2295: Rx-only 2288: 2286: 2276: 2273: 2263: 2256: 2247: 2244: 2234: 2233: 2231: 2229: 2225: 2220: 2212: 2207: 2204: 2203: 2201: 2199: 2195: 2192: 2189: 2187: 2181: 2174: 2169: 2160: 2159: 2157: 2155: 2151: 2147: 2143: 2141: 2137: 2133: 2129: 2127: 2123: 2120:Clinical data 2118: 2114: 2109: 2096: 2090: 2086: 2082: 2079: 2075: 2071: 2068: 2064: 2061: 2058: 2055: 2052: 2049: 2048: 2047: 2045: 2041: 2037: 2033: 2029: 2025: 2023: 2019: 2012: 2005: 2000: 1998: 1994: 1990: 1985: 1983: 1979: 1974: 1972: 1968: 1964: 1961: 1957: 1952: 1950: 1945: 1943: 1939: 1929: 1927: 1924: 1920: 1916: 1912: 1908: 1904: 1899: 1897: 1892: 1890: 1886: 1882: 1878: 1874: 1867: 1860: 1852: 1850: 1846: 1838: 1834: 1829: 1826: 1822: 1816: 1807: 1798: 1796: 1792: 1788: 1784: 1783:alpha helices 1780: 1776: 1772: 1767: 1763: 1761: 1757: 1753: 1749: 1745: 1741: 1737: 1732: 1730: 1723: 1722: 1717: 1713: 1709: 1706: 1703:A1AT is a 52- 1696: 1694: 1690: 1686: 1682: 1678: 1674: 1671: 1667: 1663: 1659: 1655: 1651: 1647: 1643: 1639: 1629: 1627: 1618: 1616: 1615:chromosome 14 1612: 1602: 1596: 1591: 1589: 1585: 1581: 1576: 1574: 1570: 1566: 1562: 1558: 1554: 1550: 1546: 1542: 1538: 1533: 1531: 1527: 1523: 1519: 1515: 1511: 1501: 1499: 1495: 1487: 1483: 1479: 1475: 1471: 1465: 1461: 1459: 1455: 1451: 1447: 1443: 1439: 1435: 1431: 1427: 1423: 1419: 1414: 1412: 1408: 1404: 1400: 1396: 1392: 1388: 1384: 1380: 1372: 1368: 1360: 1356: 1353: 1349: 1345: 1341: 1337: 1333: 1325: 1321: 1313: 1301: 1296: 1292: 1288: 1285: 1281: 1274: 1272: 1269: 1265: 1261: 1257: 1254: 1250: 1246: 1239: 1237: 1233: 1229: 1223: 1219: 1216: 1210: 1208: 1204: 1200: 1196: 1192: 1188: 1182: 1178: 1174: 1170: 1166: 1162: 1158: 1155: 1151: 1144: 1142: 1136: 1132: 1129: 1123: 1121: 1117: 1113: 1109: 1105: 1101: 1095: 1091: 1087: 1083: 1079: 1075: 1071: 1068: 1066:RefSeq (mRNA) 1064: 1057: 1056: 1052: 1047: 1043: 1040: 1034: 1033: 1028: 1024: 1021: 1019: 1015: 1008: 1007: 1002: 998: 995: 989: 988: 984: 979: 975: 972: 970: 966: 959: 958: 953: 949: 946: 940: 939: 934: 930: 927: 925: 921: 918: 915: 913: 910: 906: 903: 899: 895: 888: 884: 879: 873: 870: 868: 865: 863: 860: 858: 855: 853: 850: 848: 845: 843: 840: 838: 835: 833: 830: 828: 825: 824: 822: 819: 818: 812: 809: 807: 804: 802: 799: 797: 794: 792: 789: 787: 784: 782: 779: 777: 774: 772: 769: 767: 764: 762: 759: 757: 754: 752: 749: 748: 746: 743: 742: 736: 733: 731: 728: 726: 723: 721: 718: 716: 713: 712: 710: 707: 706: 703: 702:Gene ontology 699: 695: 683: 678: 674: 667: 662: 659: 657: 653: 645: 640: 629: 625: 621: 618:lobe of liver 617: 613: 609: 605: 601: 597: 593: 592: 589: 585: 580: 577: 567: 563: 559: 555: 551: 547: 543: 539: 535: 531: 530: 527: 523: 518: 515: 514: 511: 509: 505: 503: 502: 498: 497: 494: 492: 488: 484: 480: 476: 468: 463: 459: 455: 450: 440: 436: 429: 422: 416: 409: 401: 397: 393: 388: 384: 379: 375: 367: 362: 358: 354: 349: 339: 335: 328: 321: 315: 308: 304: 298: 294: 290: 285: 281: 276: 272: 268: 264: 260: 256: 252: 248: 244: 240: 236: 232: 224: 219: 212: 207: 202: 191: 189: 185: 181: 177: 173: 169: 165: 161: 157: 153: 149: 145: 141: 137: 133: 129: 125: 121: 117: 113: 109: 105: 101: 97: 93: 89: 83: 78: 75: 74: 70: 67: 60: 56: 51: 47: 43: 38: 33: 30: 19: 6196:Orphan drugs 6121:C1-inhibitor 6103: 6079: 5977:Avatrombopag 5964: 5960:Fostamatinib 5917: 5911:Catridecacog 5902:I/Fibrinogen 5888: 5882:Eptacog alfa 5874: 5867:Nonacog alfa 5819: 5796:Menadione (K 5542: 5151:Antithrombin 5043: 4788:C1-inhibitor 4783:Antithrombin 4709: 4694: 4679: 4664: 4649: 4634: 4619: 4604: 4589: 4574: 4559: 4544: 4529: 4514: 4499: 4440: 4434: 4426: 4366: 4362: 4323: 4319: 4290: 4286: 4251: 4247: 4208: 4204: 4175: 4171: 4149:(2): 89–92. 4146: 4142: 4112:(1): 33–41. 4109: 4105: 4080: 4076: 4051: 4047: 4006: 4002: 3977: 3973: 3940: 3936: 3898: 3894: 3884: 3850:(12): 2754. 3847: 3843: 3833: 3801:(3): 272–9. 3798: 3794: 3784: 3749: 3745: 3735: 3710: 3706: 3700: 3678:. Retrieved 3666: 3634:. Retrieved 3621: 3597:. Retrieved 3584: 3560:. Retrieved 3547: 3523:. Retrieved 3510: 3486:. Retrieved 3473: 3449:. Retrieved 3436: 3433:"Aralast NP" 3412:. Retrieved 3403: 3394: 3382:. Retrieved 3377: 3368: 3356:. Retrieved 3349: 3340: 3328:. Retrieved 3323: 3314: 3302:. Retrieved 3293: 3284: 3272:. Retrieved 3263: 3254: 3242:. Retrieved 3233: 3224: 3197: 3191: 3166: 3162: 3155: 3130: 3126: 3120: 3093: 3089: 3079: 3044: 3040: 3030: 3003: 2999: 2989: 2956: 2952: 2935: 2928:. Retrieved 2919: 2910: 2875: 2871: 2861: 2826: 2822: 2812: 2777: 2773: 2763: 2738: 2734: 2728: 2719: 2710: 2701: 2692: 2610: 2597: 2594: 2590: 2582:blood plasma 2579: 2568:   2228:Legal status 2222:Legal status 2154:License data 2099:Medical uses 2094: 2027: 2026: 2014: 2007: 2001: 1997:heterozygote 1991:of the A1AT 1986: 1977: 1975: 1953: 1949:turbidimetry 1946: 1935: 1911:carbamylated 1900: 1893: 1873:antithrombin 1862: 1855: 1853: 1818: 1768: 1764: 1733: 1725: 1719: 1715: 1702: 1635: 1619: 1608: 1594: 1592: 1579: 1577: 1564: 1560: 1559:, the third 1556: 1548: 1536: 1534: 1525: 1507: 1504:Nomenclature 1466: 1462: 1415: 1407:inflammatory 1382: 1374: 1370: 1362: 1351: 1339: 1335: 1327: 1323: 1320:-antitrypsin 1315: 1311: 1310: 1232:NP_001239498 1225: 1207:NP_001121179 1203:NP_001121178 1199:NP_001121177 1195:NP_001121176 1191:NP_001121175 1187:NP_001121174 1184: 1177:NP_001121173 1173:NP_001121172 1169:NP_001002236 1165:NP_001002235 1138: 1120:NM_001127706 1116:NM_001127705 1112:NM_001127704 1108:NM_001127703 1104:NM_001127702 1100:NM_001127701 1097: 1090:NM_001127700 1086:NM_001002236 1082:NM_001002235 1074:NM_001127707 1049: 1030: 1004: 981: 955: 936: 916: 911: 610:human kidney 606:right kidney 560:human kidney 506: 499: 465:103,739,851 452:103,729,853 226:External IDs 85: 29: 6136:Ulinastatin 6081:amino acids 6038:Fibrin glue 6029:Epinephrine 5982:Eltrombopag 5972:Romiplostim 5897:II/Thrombin 5810:Coagulation 5762:coagulation 5702:Transferrin 5681:Orosomucoid 5666:Haptoglobin 5510:Parvalbumin 5500:Lactalbumin 5392:Lactoferrin 5343:Fibronectin 5300:Plasminogen 5280:Angiostatin 5261:Transferrin 5221:Haptoglobin 5210:Orosomucoid 5178:Transcortin 5018:Transcortin 4482:PDB gallery 4178:(2): 93–8. 3544:"Prolastin" 3000:Exp Hematol 2635:Orosomucoid 2601:Recombinant 2562: g·mol 2495:100.029.919 2306:Identifiers 2191:Intravenous 2126:Trade names 2020:stands for 1936:A1AT has a 1909:toward the 1875:(a related 1779:beta sheets 1617:(14q32.1). 1494:Recombinant 552:gallbladder 548:granulocyte 364:94,390,693 351:94,376,747 204:Identifiers 6175:Categories 6033:adrenalone 5955:Etamsylate 5950:Concizumab 5940:Batroxobin 5875:extrinsic: 5820:intrinsic: 5479:Prealbumin 5441:Conalbumin 4761:inhibitory 4363:Hepatology 3216:1164833220 2953:Proteomics 2930:13 October 2687:, May 2017 2666:, May 2017 2642:References 2551:Molar mass 2428:PDB ligand 2397:F43I396OIS 2372:ChemSpider 2332:CAS Number 2315:IUPAC name 2261:Schedule D 2040:polymerise 2036:proteasome 2013:MM, where 2004:blood test 1973:gradient. 1919:arthralgia 1869:Pittsburgh 1837:methionine 1791:polymerise 1740:covalently 1650:Asparagine 1638:glycoforms 1590:1 region. 1514:covalently 1440:, such as 1430:elasticity 1389:(not just 827:hemostasis 510:(ortholog) 247:HomoloGene 6132:unsorted 6111:Aprotinin 5779:Vitamin K 5676:Hemopexin 5446:Ovalbumin 5433:Egg white 5305:Properdin 5285:Hemopexin 5215:alpha-2 ( 5208:alpha-1 ( 5183:alpha-2 ( 5176:alpha-1 ( 5098:globulins 5044:see also 5013:Ovalbumin 4907:SERPINB13 4902:SERPINB10 4850:SERPINA14 3618:"Zemaira" 3507:"Glassia" 3470:"Aralast" 3234:Drugs.com 3169:: 77–84. 2341:9041-92-3 2184:Routes of 2146:Monograph 2140:Drugs.com 2085:glutamate 2074:glutamate 1956:phenotype 1926:biomarker 1923:antigenic 1861:, termed 1787:Mutations 1769:Like all 1685:UniProtKB 1654:UniProtKB 1632:Structure 1626:Europeans 1573:globulins 1535:The term 1518:peptidase 1512:in vitro 1454:cirrhosis 1416:When the 1387:proteases 1236:NP_033269 1228:NP_033272 1161:NP_000286 1141:NM_009246 1078:NM_000295 902:Orthologs 255:GeneCards 6165:Medicine 6126:Camostat 6053:Thrombin 6024:Collagen 5933:systemic 5771:Systemic 5690:Negative 5656:Ferritin 5543:see also 5424:Albumins 5295:Factor H 4959:SERPINI2 4954:SERPINI1 4949:SERPINH1 4944:SERPING1 4939:SERPINF1 4934:SERPINE2 4929:SERPINE2 4924:SERPINE1 4919:SERPIND1 4914:SERPINC1 4897:SERPINB9 4892:SERPINB8 4887:SERPINB7 4882:SERPINB6 4877:SERPINB5 4872:SERPINB4 4867:SERPINB3 4862:SERPINB2 4857:SERPINB1 4845:SERPINA9 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881:Sources: 622:yolk sac 568:appendix 556:duodenum 544:monocyte 343:14q32.13 259:SERPINA1 218:SERPINA1 35:SERPINA1 18:SERPINA1 6105:serpins 5889:common: 5812:factors 5597:Amyloid 5367:Edestin 5141:alpha-2 5127:alpha-1 5117:serpins 4754:Serpins 4413:I04.001 4341:4952210 4134:2458903 4068:9466920 4031:7045697 4011:Bibcode 3994:2696185 3957:2669992 3917:4158556 3908:1932630 3867:9784676 3816:7857706 3767:6373587 3384:1 April 3274:9 April 3147:6604220 3062:1746953 2893:5473067 2844:2213124 2795:4298394 2685:Ensembl 2664:Ensembl 2617:allelic 2613:Laurell 2607:History 2512:Formula 2361:DB00058 2257: / 2253:: 2214:) 2208: ( 2206:B02AB02 2170::  1960:protein 1760:elastin 1736:enzymes 1677:protein 1673:epitope 1658:glycans 1646:glycans 1642:glycans 1586:of the 1584:protein 1553:albumin 1537:alpha-1 1510:trypsin 1432:in the 1426:elastin 1403:enzymes 1399:tissues 1391:trypsin 1344:protein 1342:) is a 1018:UniProt 969:Ensembl 908:Species 887:QuickGO 614:Hindgut 485:pattern 211:Aliases 6151:Portal 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The 1348:serpin 1270:search 1268:PubMed 1055:P07758 1051:Q00897 1032:P01009 924:Entrez 656:BioGPS 626:midgut 243:891968 235:107400 6007:Local 5931:Other 5515:Ricin 5488:Other 5336:Other 5095:Serum 4389:S2CID 4130:S2CID 4035:S2CID 3961:S2CID 3707:Chest 3670:(EMA) 3626:(FDA) 3622:U.S. 3589:(FDA) 3585:U.S. 3552:(FDA) 3548:U.S. 3515:(FDA) 3511:U.S. 3478:(FDA) 3474:U.S. 3441:(FDA) 3437:U.S. 3404:(emc) 2977:S2CID 2435:KB2 ( 1958:. 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A 1355:gene 1324:A1AT 938:5265 491:Bgee 439:Band 400:Chr. 338:Band 297:Chr. 231:OMIM 188:5IO1 184:4PYW 180:9API 176:8API 172:7API 168:3T1P 164:3NE4 160:3NDF 156:3NDD 152:3DRU 148:3DRM 144:3CWM 140:3CWL 136:2QUG 132:2D26 128:1QMB 124:1QLP 120:1PSI 116:1OPH 112:1OO8 108:1KCT 104:1IZ2 100:1HP7 96:1EZX 92:1D5S 88:1ATU 69:RCSB 66:PDBe 5878:VII 5748:B02 5610:SAA 5605:SAP 4371:doi 4336:PMC 4328:doi 4295:doi 4291:352 4264:PMC 4256:doi 4252:110 4221:PMC 4213:doi 4209:110 4180:doi 4151:doi 4114:doi 4110:321 4085:doi 4056:doi 4052:275 4019:doi 4007:298 3982:doi 3945:doi 3903:PMC 3862:PMC 3852:doi 3811:PMC 3803:doi 3762:PMC 3754:doi 3715:doi 3711:117 3171:doi 3135:doi 3131:309 3098:doi 3094:275 3057:PMC 3049:doi 3008:doi 2961:doi 2888:PMC 2880:doi 2839:PMC 2831:doi 2827:201 2790:PMC 2782:doi 2778:179 2743:doi 2739:102 2560:.65 2558:324 2538:601 2532:514 2461:EPA 2272:POM 2211:WHO 2087:to 2076:to 2002:In 1944:. 1896:PAS 1885:Arg 1883:358 1881:Met 1777:of 1705:kDa 1405:of 1340:AAT 1336:A1A 1314:or 461:End 360:End 263:OMA 239:MGI 59:PDB 6177:: 5865:, 5861:/( 5859:IX 5851:, 5847:, 5843:, 5839:, 5835:, 5831:, 5827:/( 5651:C3 5219:, 5133:, 4387:. 4379:. 4367:42 4365:. 4361:. 4344:. 4334:. 4322:. 4318:. 4301:. 4289:. 4272:. 4262:. 4250:. 4246:. 4229:. 4219:. 4207:. 4203:. 4186:. 4176:30 4174:. 4157:. 4147:30 4145:. 4128:. 4120:. 4108:. 4091:. 4079:. 4062:. 4050:. 4033:. 4025:. 4017:. 4005:. 3988:. 3976:. 3959:. 3951:. 3939:. 3911:. 3899:17 3897:. 3893:. 3870:. 3860:. 3848:14 3846:. 3842:. 3819:. 3809:. 3797:. 3793:. 3770:. 3760:. 3748:. 3744:. 3721:. 3709:. 3665:. 3646:^ 3620:. 3609:^ 3583:. 3572:^ 3546:. 3535:^ 3509:. 3498:^ 3472:. 3461:^ 3435:. 3424:^ 3402:. 3376:. 3348:. 3322:. 3292:. 3262:. 3232:. 3210:. 3177:. 3167:80 3165:. 3141:. 3129:. 3106:. 3092:. 3088:. 3065:. 3055:. 3045:59 3043:. 3039:. 3016:. 3004:30 3002:. 2998:. 2975:. 2967:. 2955:. 2943:^ 2934:. 2918:. 2896:. 2886:. 2876:44 2874:. 2870:. 2847:. 2837:. 2825:. 2821:. 2798:. 2788:. 2776:. 2772:. 2749:. 2737:. 2718:. 2700:. 2671:^ 2650:^ 2556:44 2544:10 2441:, 2291:EU 2279:US 2266:UK 2250:CA 2243:S4 2237:AU 2163:US 1971:pH 1891:. 1851:. 1785:. 1762:. 1695:. 1597:(α 1575:. 1460:. 1413:. 1334:, 1332:AT 1326:, 885:/ 467:bp 454:bp 366:bp 353:bp 261:; 257:: 253:; 249:: 245:; 241:: 237:; 233:: 186:, 182:, 178:, 174:, 170:, 166:, 162:, 158:, 154:, 150:, 146:, 142:, 138:, 134:, 130:, 126:, 122:, 118:, 114:, 110:, 106:, 102:, 98:, 94:, 90:, 6153:: 6031:/ 5909:/ 5892:X 5880:/ 5869:) 5855:) 5800:) 5798:3 5791:) 5789:1 5764:) 5760:( 5750:) 5746:( 5736:e 5729:t 5722:v 5581:e 5574:t 5567:v 5506:) 5502:( 5355:/ 5349:) 5248:: 5223:) 5212:) 5187:) 5180:) 5169:: 5157:) 5153:( 5147:) 5143:( 5137:) 5129:( 5119:: 5079:e 5072:t 5065:v 4746:e 4739:t 4732:v 4475:e 4468:t 4461:v 4448:. 4395:. 4373:: 4352:. 4330:: 4324:5 4309:. 4297:: 4280:. 4258:: 4237:. 4215:: 4194:. 4182:: 4165:. 4153:: 4136:. 4116:: 4099:. 4087:: 4070:. 4058:: 4041:. 4021:: 4013:: 3996:. 3984:: 3978:5 3967:. 3947:: 3941:9 3919:. 3878:. 3854:: 3827:. 3805:: 3799:7 3778:. 3756:: 3750:6 3729:. 3717:: 3695:. 3684:. 3640:. 3603:. 3566:. 3529:. 3492:. 3455:. 3418:. 3388:. 3362:. 3334:. 3308:. 3278:. 3248:. 3218:. 3185:. 3173:: 3149:. 3137:: 3114:. 3100:: 3073:. 3051:: 3024:. 3010:: 2983:. 2963:: 2957:6 2904:. 2882:: 2855:. 2833:: 2806:. 2784:: 2757:. 2745:: 2722:. 2704:. 2541:S 2535:O 2529:N 2523:H 2517:C 2463:) 2459:( 2447:) 2293:: 2281:: 2268:: 2239:: 2138:/ 2069:) 2017:i 2015:P 2010:i 2008:P 1978:M 1865:i 1863:P 1858:i 1856:P 1841:1 1728:i 1726:P 1724:( 1622:1 1599:1 1567:( 1490:1 1381:( 1377:1 1369:( 1365:1 1330:1 1328:α 1322:( 1318:1 1316:α 385:) 282:) 265:: 20:)

Index

SERPINA1

PDB
PDBe
RCSB
1ATU
1D5S
1EZX
1HP7
1IZ2
1KCT
1OO8
1OPH
1PSI
1QLP
1QMB
2D26
2QUG
3CWL
3CWM
3DRM
3DRU
3NDD
3NDF
3NE4
3T1P
7API
8API
9API
4PYW

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