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Perforin-1

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macrophage activation syndrome, particularly in patients with juvenile rheumatoid arthritis. Onset of symptoms usually occurs after the age of 5. Moreover, some research suggests a correlation between variations in the PRF1 gene and the outcome of allogeneic bone marrow transplantation. However, these associations remain controversial, with studies disproving the connection outweighing those supporting it.
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Rosado CJ, Buckle AM, Law RH, Butcher RE, Kan WT, Bird CH, Ung K, Browne KA, Baran K, Bashtannyk-Puhalovich TA, Faux NG, Wong W, Porter CJ, Pike RN, Ellisdon AM, Pearce MC, Bottomley SP, Emsley J, Smith AI, Rossjohn J, Hartland EL, Voskoboinik I, Trapani JA, Bird PI, Dunstone MA, Whisstock JC (2007).
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via membrane phospholipids while phosphatidylcholine binds calcium ions to increase perforin's affinity to the membrane. Perforin oligomerises in a Ca2+ dependent manner to form pores on the target cell. The pore formed allows for the passive diffusion of a family of pro-apoptotic proteases, known as
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Chronic perforinopathies are regarded as a range of immune-related conditions resulting from monoallelic mutations in genes linked to familial hemophagocytic lymphohistiocytosis (FHL). These conditions typically manifest differently from classic FHL and may include conditions like blood cancers and
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Sub-acute perforinopathies encompass a diverse array of symptoms, all stemming from a partial ("sub-total") reduction in cytotoxic lymphocyte (CL) activity due to bi-allelic mutations in one of the four previously mentioned genes. In contrast to the acute form, diagnosing sub-acute perforinopathies
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and is capable of lysing non-specifically a variety of target cells. This protein is one of the main cytolytic proteins of cytolytic granules, and it is known to be a key effector molecule for T-cell- and natural killer-cell-mediated cytolysis. Perforin is thought to act by creating holes in the
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could be induced by adding perforin to washed cells that had already endocytosed granzyme B, even in the absence of perforin during the endocytosis process. In light of these findings, Froelich and colleagues suggested that perforin's action might not occur at the plasma membrane as previously
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Perforin was initially discovered in 1983 and subsequently cloned from an expression library in 1988 using anti-complement C9 antibody cross-reactivity. A sequence comparison showed a notable resemblance between the two proteins in a specific central region, termed the 'membrane attack
1171:(FHL) and meet most or all of the criteria outlined in HLH-2004. Diagnosis is confirmed by reduced NK cell cytotoxicity, as healthy NK cells normally exhibit constitutive, pathogen-independent cytotoxicity, and by identifying mutations in genes such as PRF1, UNC13D, STX11, and STXBP2. 1183:
can be challenging due to their typically milder and more sporadic clinical manifestations, an intermittent disease course, variability in onset age, and their frequent positive response to non-specific immune-suppressive or immune-ablative treatments.
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Perforin (PRF), encoded by the PRF1 gene, is a pore-forming toxic protein housed in the secretory granules of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. Together, these cells are known as cytotoxic lymphocytes (CLs).
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Young JD, Cohn ZA, Podack ER (1986). "The ninth component of complement and the pore-forming protein (perforin 1) from cytotoxic T cells: structural, immunological, and functional similarities".
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Froelich, Christopher J.; Orth, Kim; Turbov, Jane; Seth, Prem; Gottlieb, Roberta; Babior, Bernard; Shah, Girish M.; Bleackley, R. Christopher; Dixit, Vishva M.; Hanna, William (November 1996).
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Young JD, Hengartner H, Podack ER, Cohn ZA (1986). "Purification and characterization of a cytolytic pore-forming protein from granules of cloned lymphocytes with natural killer activity".
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Around the turn of the 21st century, it was recognized that a total loss of perforin activity leads to a severe, fatal autosomal recessive immunoregulatory disorder in infants, known as
1807:(1996). "Target cell apoptosis induced by cytotoxic T cells and natural killer cells involves synergy between the pore-forming protein, perforin, and the serine protease, granzyme B". 1505:(1996). "Target cell apoptosis induced by cytotoxic T cells and natural killer cells involves synergy between the pore-forming protein, perforin, and the serine protease, granzyme B". 1838:
Peitsch MC, Amiguet P, Guy R, et al. (1990). "Localization and molecular modelling of the membrane-inserted domain of the ninth component of human complement and perforin".
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thought, but rather at the endosomal membrane. They proposed that perforin likely facilitates the release of granzymes from endosomes by forming pores in the endosomal membrane.
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Andrin C, Pinkoski MJ, Burns K, et al. (July 1998). "Interaction between a Ca2+-binding protein calreticulin and perforin, a component of the cytotoxic T-cell granules".
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Andrin C, Pinkoski MJ, Burns K, et al. (1998). "Interaction between a Ca2+-binding protein calreticulin and perforin, a component of the cytotoxic T-cell granules".
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plasma membrane which triggers an influx of calcium and initiates membrane repair mechanisms. These repair mechanisms bring perforin and granzymes into early endosomes.
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Purifying perforin is challenging due to its tendency to lose activity and stability in solution, and only recently has a recombinant form been successfully produced.
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Goebel WS, Schloemer RH, Brahmi Z (1996). "Target cell-induced perforin mRNA turnover in NK3.3 cells is mediated by multiple elements within the mRNA coding region".
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Osińska, Iwona et al. “Perforin: an important player in immune response.” Central-European journal of immunology vol. 39,1 (2014): 109-15. doi:10.5114/ceji.2014.42135
1160:(FHL), typically appearing before 12 months of age. This condition can only be treated effectively with a bone marrow transplant from a non-related donor. 2410:
Ambach A, Bonnekoh B, Gollnick H (2001). "Perforin granule release from cytotoxic lymphocytes ex vivo is inhibited by ciclosporin but not by methotrexate".
1543:; Masson D; Stanley KK (1986). "Structural/functional similarity between proteins involved in complement- and cytotoxic T-lymphocyte-mediated cytolysis". 784: 1167:
and CTLs to present functional perforin, thereby failing to kill target cells. Infants affected by this condition typically show symptoms of “classic”
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Badovinac VP, Tvinnereim AR, Harty JT (2000). "Regulation of antigen-specific CD8+ T cell homeostasis by perforin and interferon-gamma".
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Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. (1999). "Perforin gene defects in familial hemophagocytic lymphohistiocytosis".
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Thiery J, Keefe D, Boulant S, Boucrot E, Walch M, Martinvalet D, Goping IS, Bleackley RC, Kirchhausen T, Lieberman J (2011).
1275: 2252:"Analysis of human V alpha 24+ CD4+ NKT cells activated by alpha-glycosylceramide-pulsed monocyte-derived dendritic cells" 1717:"Perforin pores in the endosomal membrane trigger the release of endocytosed granzyme B into the cytosol of target cells" 1254: 1230: 1168: 1157: 264: 2639: 2493: 241: 2105:"Virus-Specific CD4+ T Cells Eliminate Borna Disease Virus from the Brain via Induction of Cytotoxic CD8+ T Cells" 1940:"Structure of the human perforin gene. A simple gene organization with interesting potential regulatory sequences" 1095:, which works as a chaperone protein to prevent perforin from degrading. Perforin then binds to the target cell's 1279: 1258: 2659: 2522: 829: 1804: 1502: 163: 138: 810: 2486: 80: 1204: 1982:
Shinkai Y, Takio K, Okumura K (1988). "Homology of perforin to the ninth component of complement (C9)".
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Lichtenheld MG, Olsen KJ, Lu P, et al. (1988). "Structure and function of human perforin".
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The initial concept of a plasma membrane pore model was challenged when research revealed that
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The pathogenesis involves a sequence of downstream events resulting from the inability of
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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
2372: 1870: 433: 2478: 2180:"Role of a STAT binding site in the regulation of the human perforin promoter" 1463: 2648: 1840: 1679: 1670: 1653: 1471: 1421: 1397: 1364: 1355: 1104:, into the target cell. The lytic membrane-inserting part of perforin is the 1088: 544: 2634: 1922: 1621: 1596: 1448:"Delivering the kiss of death: progress on understanding how perforin works" 493: 371: 2530: 2431: 2402: 2358: 2314: 2277: 2242: 2205: 1750: 1630: 1479: 1429: 1382: 1208: 1092: 515: 350: 129: 2170: 2139: 2095: 2058: 2013: 1966: 1930: 1891: 1860: 1830: 1785: 1687: 1572: 1526: 1039: 1034: 2621: 2596: 2384: 1944: 1413: 1120: 874: 855: 2322:
Göransdotter Ericson K, Fadeel B, Nilsson-Ardnor S, et al. (2001).
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independently of perforin. Moreover, experiments demonstrated that
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Brennan, A J; Chia, J; Trapani, J A; Voskoboinik, I (April 2010).
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domain. This region shares homology with cholesterol-dependent
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positive regulation of killing of cells of other organism
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Clementi R, zur Stadt U, Savoldi G, et al. (2002).
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Perforin has structural and functional similarities to
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Takahashi T, Nieda M, Koezuka Y, et al. (2000).
2249: 2102: 2073: 1702:"Entrez Gene: PRF1 perforin 1 (pore forming protein)" 505: 2146: 1981: 1763: 2178:Yu CR, Ortaldo JR, Curiel RE, et al. (1999). 2028: 1398:"Perforin deficiency and susceptibility to cancer" 1336: 1271: 1269: 1267: 1250: 1248: 1246: 1135:(C9). Like C9, this protein creates transmembrane 1937: 1837: 1446:Pipkin, Matthew E; Lieberman, Judy (2007-06-01). 287: 186: 2646: 2508: 2177: 1906: 1445: 1264: 1243: 1810:Australian and New Zealand Journal of Medicine 1587: 1507:Australian and New Zealand Journal of Medicine 1337:Voskoboinik, Ilia; Trapani, Joseph A. (2013). 1276:GRCm38: Ensembl release 89: ENSMUSG00000037202 2494: 2452:: CS1 maint: DOI inactive as of June 2024 ( 2103:Nöske K, Bilzer T, Planz O, Stitz L (1998). 1708: 1757: 1255:GRCh38: Ensembl release 89: ENSG00000180644 1169:familial hemophagocytic lymphohistiocytosis 1158:familial hemophagocytic lymphohistiocytosis 2501: 2487: 1803: 1501: 1495: 2471:at the U.S. National Library of Medicine 2392: 2348: 2267: 2195: 2129: 1956: 1740: 1669: 1620: 1372: 1354: 1067: 1143: 71:, FLH2, HPLH2, P1, PFN1, PFP, perforin 1 27:Mammalian protein found in Homo sapiens 14: 2647: 1694: 2482: 1152:presentations of perforinopathy — FHL 292: 253: 248: 191: 150: 145: 1441: 1439: 1332: 1330: 1328: 1326: 1324: 1064:complex/perforin' (MACPF) domain. 24: 2412:Skin Pharmacol. Appl. Skin Physiol 1938:Lichtenheld MG, Podack ER (1990). 1823:10.1111/j.1445-5994.1995.tb02883.x 1796: 1519:10.1111/j.1445-5994.1995.tb02883.x 1231:Complement membrane attack complex 25: 2676: 2462: 1436: 1321: 1079:protein found in the granules of 1402:Cell Death & Differentiation 514: 456:lumbar subsegment of spinal cord 276: 269: 263: 240: 175: 168: 162: 137: 45: 2523:Antimicrobial cationic peptides 2122:10.1128/JVI.72.5.4387-4395.1998 1658:Journal of Biological Chemistry 1645: 1533: 1198: 1190:presentations of perforinopathy 1178:presentations of perforinopathy 1085:natural killer cells (NK cells) 655:immunological synapse formation 1486: 1389: 1303: 1285: 1081:cytotoxic T lymphocytes (CTLs) 680:defense response to tumor cell 594:integral component of membrane 525:More reference expression data 494:More reference expression data 13: 1: 2307:10.1126/science.290.5495.1354 2235:10.1126/science.286.5446.1957 1452:Current Opinion in Immunology 1236: 1112:from Gram-positive bacteria. 685:immune response to tumor cell 261: 160: 2655:Genes on human chromosome 10 2088:10.1016/0161-5890(95)00155-7 1958:10.4049/jimmunol.143.12.4267 1884:10.1016/0092-8674(86)90007-3 1853:10.1016/0161-5890(90)90001-G 1058: 700:T cell mediated cytotoxicity 7: 2269:10.4049/jimmunol.164.9.4458 2197:10.4049/jimmunol.162.5.2785 1214: 1203:Perforin has been shown to 1075:Perforin is a pore forming 690:protein homooligomerization 472:subcutaneous adipose tissue 414:superficial temporal artery 10: 2681: 573:wide pore channel activity 29: 2579: 2563: 2521: 1464:10.1016/j.coi.2007.04.011 1311:"Mouse PubMed Reference:" 1293:"Human PubMed Reference:" 1038: 1033: 1029: 1022: 1006: 987: 972: 968: 947: 943: 936: 921: 917: 896: 892: 885: 872: 868: 853: 849: 840: 827: 823: 808: 804: 795: 782: 778: 763: 759: 750: 735: 728: 724: 708: 695:defense response to virus 650:cellular defense response 578:identical protein binding 543: 539: 522: 513: 504: 491: 440: 431: 378: 369: 339: 331: 327: 310: 297: 260: 239: 230: 226: 209: 196: 159: 136: 127: 123: 78: 75: 65: 58: 53: 44: 39: 2642:Genetics Home Reference 2473:Medical Subject Headings 1671:10.1074/jbc.271.46.29073 1356:10.3389/fimmu.2013.00441 1000:Chr 10: 61.13 – 61.14 Mb 30:Not to be confused with 2426:(inactive 2024-06-11). 1923:10.1126/science.2425429 1622:10.1126/science.1144706 1343:Frontiers in Immunology 670:transmembrane transport 418:upper lobe of left lung 1133:complement component 9 1068:Structure and Function 993:Chr 10: 70.6 – 70.6 Mb 294:10 B4|10 32.18 cM 2660:Programmed cell death 1144:Clinical Significance 255:Chromosome 10 (mouse) 153:Chromosome 10 (human) 2385:10.1136/jmg.38.9.643 1414:10.1038/cdd.2009.212 599:extracellular region 2510:Pore-forming toxins 2299:2000Sci...290.1354B 2043:1988Natur.335..448L 1998:1988Natur.334..525S 1664:(46): 29073–29079. 1613:2007Sci...317.1548R 1557:1986Natur.322..831T 629:cytoplasmic vesicle 568:calcium ion binding 2329:Am. J. Hum. Genet. 830:ENSMUSG00000037202 643:Biological process 587:Cellular component 551:Molecular function 402:buccal mucosa cell 2630: 2629: 2424:10.1159/000056355 2163:10.1021/bi980595z 1778:10.1021/bi980595z 1607:(5844): 1548–51. 1049: 1048: 1045: 1044: 1018: 1017: 983: 982: 962: 961: 932: 931: 911: 910: 881: 880: 862: 861: 836: 835: 817: 816: 791: 790: 772: 771: 720: 719: 665:apoptotic process 634:cytolytic granule 563:metal ion binding 535: 534: 531: 530: 500: 499: 487: 486: 425: 424: 398:bone marrow cells 323: 322: 222: 221: 16:(Redirected from 2672: 2592:Diphtheria toxin 2503: 2496: 2489: 2480: 2479: 2457: 2451: 2443: 2406: 2396: 2362: 2352: 2318: 2293:(5495): 1354–8. 2281: 2271: 2246: 2229:(5446): 1957–9. 2217: 2199: 2174: 2157:(29): 10386–94. 2143: 2133: 2099: 2070: 2051:10.1038/335448a0 2037:(6189): 448–51. 2025: 2006:10.1038/334525a0 1978: 1960: 1934: 1917:(4760): 184–90. 1903: 1864: 1834: 1790: 1789: 1772:(29): 10386–94. 1761: 1755: 1754: 1744: 1712: 1706: 1705: 1698: 1692: 1691: 1673: 1649: 1643: 1642: 1624: 1591: 1585: 1584: 1565:10.1038/322831a0 1537: 1531: 1530: 1499: 1493: 1490: 1484: 1483: 1443: 1434: 1433: 1393: 1387: 1386: 1376: 1358: 1334: 1319: 1318: 1307: 1301: 1300: 1289: 1283: 1273: 1262: 1252: 1031: 1030: 1002: 995: 978: 966: 965: 957: 941: 940: 937:RefSeq (protein) 927: 915: 914: 906: 890: 889: 866: 865: 847: 846: 821: 820: 802: 801: 776: 775: 757: 756: 726: 725: 541: 540: 527: 518: 511: 510: 496: 436: 434:Top expressed in 429: 428: 374: 372:Top expressed in 367: 366: 346: 345: 329: 328: 319: 306: 295: 280: 273: 267: 256: 244: 228: 227: 218: 205: 194: 179: 172: 166: 155: 141: 125: 124: 119: 117:PRF1 - orthologs 70: 63: 49: 37: 36: 21: 2680: 2679: 2675: 2674: 2673: 2671: 2670: 2669: 2645: 2644: 2631: 2626: 2580:Other, nonhuman 2575: 2559: 2517: 2507: 2465: 2460: 2445: 2444: 1992:(6182): 525–7. 1951:(12): 4267–74. 1799: 1797:Further reading 1794: 1793: 1762: 1758: 1733:10.1038/ni.2050 1713: 1709: 1700: 1699: 1695: 1650: 1646: 1592: 1588: 1551:(6082): 831–4. 1538: 1534: 1500: 1496: 1491: 1487: 1444: 1437: 1394: 1390: 1335: 1322: 1309: 1308: 1304: 1291: 1290: 1286: 1274: 1265: 1253: 1244: 1239: 1217: 1201: 1192: 1180: 1154: 1146: 1097:plasma membrane 1070: 1061: 1040:View/Edit Mouse 1035:View/Edit Human 998: 991: 988:Location (UCSC) 974: 953: 949: 923: 902: 898: 811:ENSG00000180644 704: 638: 609:plasma membrane 582: 558:protein binding 523: 492: 483: 478: 474: 470: 466: 462: 458: 454: 450: 446: 432: 421: 416: 412: 408: 404: 400: 396: 392: 388: 384: 370: 314: 301: 293: 283: 282: 281: 274: 254: 231:Gene location ( 213: 200: 192: 182: 181: 180: 173: 151: 128:Gene location ( 79: 66: 59: 35: 28: 23: 22: 15: 12: 11: 5: 2678: 2668: 2667: 2662: 2657: 2628: 2627: 2625: 2624: 2619: 2614: 2609: 2604: 2599: 2594: 2589: 2583: 2581: 2577: 2576: 2574: 2573: 2567: 2565: 2561: 2560: 2558: 2557: 2543: 2538: 2533: 2527: 2525: 2519: 2518: 2506: 2505: 2498: 2491: 2483: 2477: 2476: 2464: 2463:External links 2461: 2459: 2458: 2407: 2373:J. Med. Genet. 2363: 2341:10.1086/318796 2319: 2282: 2262:(9): 4458–64. 2247: 2218: 2190:(5): 2785–90. 2175: 2144: 2116:(5): 4387–95. 2100: 2082:(4–5): 341–9. 2071: 2026: 1979: 1935: 1904: 1865: 1847:(7): 589–602. 1835: 1800: 1798: 1795: 1792: 1791: 1756: 1707: 1693: 1644: 1586: 1532: 1494: 1485: 1458:(3): 301–308. 1435: 1408:(4): 607–615. 1388: 1320: 1302: 1284: 1263: 1241: 1240: 1238: 1235: 1234: 1233: 1228: 1223: 1216: 1213: 1200: 1197: 1191: 1185: 1179: 1173: 1153: 1147: 1145: 1142: 1119:could undergo 1069: 1066: 1060: 1057: 1047: 1046: 1043: 1042: 1037: 1027: 1026: 1020: 1019: 1016: 1015: 1013: 1011: 1004: 1003: 996: 989: 985: 984: 981: 980: 970: 969: 963: 960: 959: 945: 944: 938: 934: 933: 930: 929: 919: 918: 912: 909: 908: 894: 893: 887: 883: 882: 879: 878: 870: 869: 863: 860: 859: 851: 850: 844: 838: 837: 834: 833: 825: 824: 818: 815: 814: 806: 805: 799: 793: 792: 789: 788: 780: 779: 773: 770: 769: 761: 760: 754: 748: 747: 742: 737: 733: 732: 722: 721: 718: 717: 706: 705: 703: 702: 697: 692: 687: 682: 677: 672: 667: 662: 657: 652: 646: 644: 640: 639: 637: 636: 631: 626: 621: 616: 614:endosome lumen 611: 606: 601: 596: 590: 588: 584: 583: 581: 580: 575: 570: 565: 560: 554: 552: 548: 547: 537: 536: 533: 532: 529: 528: 520: 519: 508: 502: 501: 498: 497: 489: 488: 485: 484: 482: 481: 477: 473: 469: 465: 461: 457: 453: 449: 445: 441: 438: 437: 426: 423: 422: 420: 419: 415: 411: 407: 403: 399: 395: 391: 387: 383: 379: 376: 375: 363: 362: 354: 343: 337: 336: 333:RNA expression 325: 324: 321: 320: 312: 308: 307: 299: 296: 291: 285: 284: 275: 268: 262: 258: 257: 252: 246: 245: 237: 236: 224: 223: 220: 219: 211: 207: 206: 198: 195: 190: 184: 183: 174: 167: 161: 157: 156: 149: 143: 142: 134: 133: 121: 120: 77: 73: 72: 64: 56: 55: 51: 50: 42: 41: 26: 9: 6: 4: 3: 2: 2677: 2666: 2663: 2661: 2658: 2656: 2653: 2652: 2650: 2643: 2641: 2637: 2636: 2623: 2620: 2618: 2615: 2613: 2610: 2608: 2605: 2603: 2600: 2598: 2595: 2593: 2590: 2588: 2585: 2584: 2582: 2578: 2572: 2569: 2568: 2566: 2562: 2555: 2551: 2547: 2544: 2542: 2539: 2537: 2534: 2532: 2529: 2528: 2526: 2524: 2520: 2515: 2511: 2504: 2499: 2497: 2492: 2490: 2485: 2484: 2481: 2474: 2470: 2467: 2466: 2455: 2449: 2441: 2437: 2433: 2429: 2425: 2421: 2418:(5): 249–60. 2417: 2413: 2408: 2404: 2400: 2395: 2390: 2386: 2382: 2378: 2375: 2374: 2369: 2364: 2360: 2356: 2351: 2346: 2342: 2338: 2334: 2331: 2330: 2325: 2320: 2316: 2312: 2308: 2304: 2300: 2296: 2292: 2288: 2283: 2279: 2275: 2270: 2265: 2261: 2257: 2253: 2248: 2244: 2240: 2236: 2232: 2228: 2224: 2219: 2215: 2211: 2207: 2203: 2198: 2193: 2189: 2185: 2181: 2176: 2172: 2168: 2164: 2160: 2156: 2152: 2151: 2145: 2141: 2137: 2132: 2127: 2123: 2119: 2115: 2112: 2111: 2106: 2101: 2097: 2093: 2089: 2085: 2081: 2077: 2072: 2068: 2064: 2060: 2056: 2052: 2048: 2044: 2040: 2036: 2032: 2027: 2023: 2019: 2015: 2011: 2007: 2003: 1999: 1995: 1991: 1987: 1986: 1980: 1976: 1972: 1968: 1964: 1959: 1954: 1950: 1947: 1946: 1941: 1936: 1932: 1928: 1924: 1920: 1916: 1912: 1911: 1905: 1901: 1897: 1893: 1889: 1885: 1881: 1878:(6): 849–59. 1877: 1873: 1872: 1866: 1862: 1858: 1854: 1850: 1846: 1843: 1842: 1841:Mol. Immunol. 1836: 1832: 1828: 1824: 1820: 1816: 1812: 1811: 1806: 1802: 1801: 1787: 1783: 1779: 1775: 1771: 1767: 1760: 1752: 1748: 1743: 1738: 1734: 1730: 1726: 1722: 1718: 1711: 1703: 1697: 1689: 1685: 1681: 1677: 1672: 1667: 1663: 1659: 1655: 1648: 1640: 1636: 1632: 1628: 1623: 1618: 1614: 1610: 1606: 1602: 1598: 1590: 1582: 1578: 1574: 1570: 1566: 1562: 1558: 1554: 1550: 1546: 1542: 1536: 1528: 1524: 1520: 1516: 1512: 1508: 1504: 1498: 1489: 1481: 1477: 1473: 1469: 1465: 1461: 1457: 1453: 1449: 1442: 1440: 1431: 1427: 1423: 1419: 1415: 1411: 1407: 1403: 1399: 1392: 1384: 1380: 1375: 1370: 1366: 1362: 1357: 1352: 1348: 1344: 1340: 1333: 1331: 1329: 1327: 1325: 1316: 1312: 1306: 1298: 1294: 1288: 1281: 1277: 1272: 1270: 1268: 1260: 1256: 1251: 1249: 1247: 1242: 1232: 1229: 1227: 1224: 1222: 1219: 1218: 1212: 1210: 1206: 1196: 1189: 1184: 1177: 1172: 1170: 1166: 1161: 1159: 1151: 1141: 1138: 1134: 1129: 1126: 1122: 1118: 1113: 1111: 1107: 1103: 1098: 1094: 1090: 1089:degranulation 1086: 1082: 1078: 1073: 1065: 1056: 1053: 1041: 1036: 1032: 1028: 1025: 1021: 1014: 1012: 1009: 1005: 1001: 997: 994: 990: 986: 979: 977: 971: 967: 964: 958: 956: 952: 946: 942: 939: 935: 928: 926: 920: 916: 913: 907: 905: 901: 895: 891: 888: 886:RefSeq (mRNA) 884: 877: 876: 871: 867: 864: 858: 857: 852: 848: 845: 843: 839: 832: 831: 826: 822: 819: 813: 812: 807: 803: 800: 798: 794: 787: 786: 781: 777: 774: 768: 767: 762: 758: 755: 753: 749: 746: 743: 741: 738: 734: 731: 727: 723: 716: 712: 707: 701: 698: 696: 693: 691: 688: 686: 683: 681: 678: 676: 673: 671: 668: 666: 663: 661: 658: 656: 653: 651: 648: 647: 645: 642: 641: 635: 632: 630: 627: 625: 622: 620: 617: 615: 612: 610: 607: 605: 602: 600: 597: 595: 592: 591: 589: 586: 585: 579: 576: 574: 571: 569: 566: 564: 561: 559: 556: 555: 553: 550: 549: 546: 545:Gene ontology 542: 538: 526: 521: 517: 512: 509: 507: 503: 495: 490: 479: 475: 471: 467: 463: 459: 455: 451: 447: 443: 442: 439: 435: 430: 427: 417: 413: 409: 406:apex of heart 405: 401: 397: 393: 389: 385: 381: 380: 377: 373: 368: 365: 364: 361: 359: 355: 353: 352: 348: 347: 344: 342: 338: 334: 330: 326: 318: 313: 309: 305: 300: 290: 286: 279: 272: 266: 259: 251: 247: 243: 238: 234: 229: 225: 217: 212: 208: 204: 199: 189: 185: 178: 171: 165: 158: 154: 148: 144: 140: 135: 131: 126: 122: 118: 114: 110: 106: 102: 98: 94: 90: 86: 82: 74: 69: 62: 57: 52: 48: 43: 38: 33: 19: 2633: 2632: 2564:Other, human 2531:Cathelicidin 2448:cite journal 2415: 2411: 2379:(9): 643–6. 2376: 2371: 2335:(3): 590–7. 2332: 2327: 2290: 2286: 2259: 2255: 2226: 2222: 2187: 2183: 2154: 2150:Biochemistry 2148: 2113: 2108: 2079: 2076:Mol. Immunol 2075: 2034: 2030: 1989: 1983: 1948: 1943: 1914: 1908: 1875: 1869: 1844: 1839: 1817:(6): 793–9. 1814: 1808: 1769: 1766:Biochemistry 1765: 1759: 1727:(8): 770–7. 1724: 1721:Nat. Immunol 1720: 1710: 1696: 1661: 1657: 1647: 1604: 1600: 1589: 1548: 1544: 1535: 1513:(6): 793–9. 1510: 1506: 1497: 1488: 1455: 1451: 1405: 1401: 1391: 1346: 1342: 1314: 1305: 1296: 1287: 1209:calreticulin 1202: 1199:Interactions 1193: 1187: 1181: 1175: 1162: 1155: 1149: 1130: 1114: 1093:calreticulin 1074: 1071: 1062: 1051: 1050: 973: 951:NP_001076585 948: 922: 904:NM_001083116 897: 873: 854: 828: 809: 783: 764: 744: 739: 356: 349: 76:External IDs 2622:Pneumolysin 2597:Dermaseptin 1945:J. Immunol. 1121:endocytosis 382:granulocyte 315:61,140,459 302:61,133,612 214:70,602,759 201:70,597,348 54:Identifiers 2649:Categories 2635:Perforin-1 2256:J. Immunol 2184:J. Immunol 1805:Trapani JA 1503:Trapani JA 1282:, May 2017 1261:, May 2017 1237:References 1117:granzyme B 1110:cytolysins 1052:Perforin-1 480:right lung 468:lymph node 410:lymph node 360:(ortholog) 97:HomoloGene 2541:Dermcidin 2110:J. Virol. 1680:0021-9258 1541:Tschopp J 1472:0952-7915 1422:1350-9047 1365:1664-3224 1221:Granzymes 1176:Sub-acute 1125:apoptosis 1102:granzymes 1077:cytolytic 1059:Discovery 976:NP_035203 955:NP_005032 925:NM_011073 900:NM_005041 730:Orthologs 660:cytolysis 105:GeneCards 32:Porphyrin 2665:Proteins 2612:Melittin 2607:Magainin 2602:Latarcin 2587:Cecropin 2571:Perforin 2546:Histatin 2536:Defensin 2469:perforin 2440:30142804 2432:11586066 2403:11565555 2359:11179007 2315:11082062 2278:10779745 2243:10583959 2214:26096007 2206:10072525 1900:30182487 1751:21685908 1639:20372720 1631:17717151 1480:17433871 1430:20075937 1383:24376445 1278:– 1257:– 1226:Defensin 1215:See also 1205:interact 1165:NK cells 1024:Wikidata 709:Sources: 619:membrane 604:endosome 444:gastrula 18:Perforin 2394:1734943 2350:1274472 2295:Bibcode 2287:Science 2223:Science 2171:9671507 2140:9557729 2096:8676885 2067:4359028 2059:3419519 2039:Bibcode 2022:4348928 2014:3261391 1994:Bibcode 1975:8326644 1967:2480391 1931:2425429 1910:Science 1892:2420467 1861:2395434 1831:8770355 1786:9671507 1742:3140544 1688:8910561 1609:Bibcode 1601:Science 1581:4330219 1573:2427956 1553:Bibcode 1527:8770355 1374:3860100 1349:: 441. 1280:Ensembl 1259:Ensembl 1188:Chronic 1137:tubules 1087:. Upon 842:UniProt 797:Ensembl 736:Species 715:QuickGO 624:cytosol 448:decidua 394:decidua 335:pattern 193:10q22.1 61:Aliases 2475:(MeSH) 2438:  2430:  2401:  2391:  2357:  2347:  2313:  2276:  2241:  2212:  2204:  2169:  2138:  2131:109669 2128:  2094:  2065:  2057:  2031:Nature 2020:  2012:  1985:Nature 1973:  1965:  1929:  1898:  1890:  1859:  1829:  1784:  1749:  1739:  1686:  1678:  1637:  1629:  1579:  1571:  1545:Nature 1525:  1478:  1470:  1428:  1420:  1381:  1371:  1363:  1010:search 1008:PubMed 875:P10820 856:P14222 752:Entrez 506:BioGPS 476:thymus 464:spleen 460:embryo 390:spleen 85:170280 2617:Nisin 2514:TC 1C 2436:S2CID 2210:S2CID 2063:S2CID 2018:S2CID 1971:S2CID 1896:S2CID 1635:S2CID 1577:S2CID 1207:with 1150:Acute 1106:MACPF 785:18646 745:Mouse 740:Human 711:Amigo 452:blood 386:blood 358:Mouse 351:Human 298:Start 233:Mouse 197:Start 130:Human 93:97551 2554:HTN3 2550:HTN1 2454:link 2428:PMID 2399:PMID 2355:PMID 2311:PMID 2274:PMID 2239:PMID 2202:PMID 2167:PMID 2136:PMID 2092:PMID 2055:PMID 2010:PMID 1963:PMID 1927:PMID 1888:PMID 1871:Cell 1857:PMID 1827:PMID 1782:PMID 1747:PMID 1684:PMID 1676:ISSN 1627:PMID 1569:PMID 1523:PMID 1476:PMID 1468:ISSN 1426:PMID 1418:ISSN 1379:PMID 1361:ISSN 1100:the 1083:and 766:5551 341:Bgee 289:Band 250:Chr. 188:Band 147:Chr. 109:PRF1 101:3698 81:OMIM 68:PRF1 40:PRF1 2640:NLM 2638:at 2420:doi 2389:PMC 2381:doi 2345:PMC 2337:doi 2303:doi 2291:290 2264:doi 2260:164 2231:doi 2227:286 2192:doi 2188:162 2159:doi 2126:PMC 2118:doi 2084:doi 2047:doi 2035:335 2002:doi 1990:334 1953:doi 1949:143 1919:doi 1915:233 1880:doi 1849:doi 1819:doi 1774:doi 1737:PMC 1729:doi 1666:doi 1662:271 1617:doi 1605:317 1561:doi 1549:322 1515:doi 1460:doi 1410:doi 1369:PMC 1351:doi 311:End 210:End 113:OMA 89:MGI 2651:: 2552:, 2450:}} 2446:{{ 2434:. 2416:14 2414:. 2397:. 2387:. 2377:38 2370:. 2353:. 2343:. 2333:68 2326:. 2309:. 2301:. 2289:. 2272:. 2258:. 2254:. 2237:. 2225:. 2208:. 2200:. 2186:. 2182:. 2165:. 2155:37 2153:. 2134:. 2124:. 2114:72 2107:. 2090:. 2080:33 2078:. 2061:. 2053:. 2045:. 2033:. 2016:. 2008:. 2000:. 1988:. 1969:. 1961:. 1942:. 1925:. 1913:. 1894:. 1886:. 1876:44 1874:. 1855:. 1845:27 1825:. 1815:25 1813:. 1780:. 1770:37 1768:. 1745:. 1735:. 1725:12 1723:. 1719:. 1682:. 1674:. 1660:. 1656:. 1633:. 1625:. 1615:. 1603:. 1599:. 1575:. 1567:. 1559:. 1547:. 1521:. 1511:25 1509:. 1474:. 1466:. 1456:19 1450:. 1438:^ 1424:. 1416:. 1406:17 1404:. 1400:. 1377:. 1367:. 1359:. 1345:. 1341:. 1323:^ 1313:. 1295:. 1266:^ 1245:^ 1211:. 713:/ 317:bp 304:bp 216:bp 203:bp 111:; 107:: 103:; 99:: 95:; 91:: 87:; 83:: 2556:) 2548:( 2516:) 2512:( 2502:e 2495:t 2488:v 2456:) 2442:. 2422:: 2405:. 2383:: 2361:. 2339:: 2317:. 2305:: 2297:: 2280:. 2266:: 2245:. 2233:: 2216:. 2194:: 2173:. 2161:: 2142:. 2120:: 2098:. 2086:: 2069:. 2049:: 2041:: 2024:. 2004:: 1996:: 1977:. 1955:: 1933:. 1921:: 1902:. 1882:: 1863:. 1851:: 1833:. 1821:: 1788:. 1776:: 1753:. 1731:: 1704:. 1690:. 1668:: 1641:. 1619:: 1611:: 1583:. 1563:: 1555:: 1529:. 1517:: 1482:. 1462:: 1432:. 1412:: 1385:. 1353:: 1347:4 1317:. 1299:. 235:) 132:) 115:: 34:. 20:)

Index

Perforin
Porphyrin

Aliases
PRF1
OMIM
170280
MGI
97551
HomoloGene
3698
GeneCards
PRF1
OMA
PRF1 - orthologs
Human
Chromosome 10 (human)
Chr.
Chromosome 10 (human)
Chromosome 10 (human)
Genomic location for PRF1
Genomic location for PRF1
Band
bp
bp
Mouse
Chromosome 10 (mouse)
Chr.
Chromosome 10 (mouse)
Genomic location for PRF1

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