Knowledge (XXG)

ADAMTS13

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Matsukawa M, Kaikita K, Soejima K, Fuchigami S, Nakamura Y, Honda T, Tsujita K, Nagayoshi Y, Kojima S, Shimomura H, Sugiyama S, Fujimoto K, Yoshimura M, Nakagaki T, Ogawa H (September 2007). "Serial changes in von Willebrand factor-cleaving protease (ADAMTS13) and prognosis after acute myocardial
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at 1606 by a plasma metalloprotease enzyme when it was exposed to high levels of shear stress. In 1996, two research groups independently further characterized this enzyme. In the next two years, the same two groups showed that the congenital deficiency of a vWF-cleaving
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Furlan M, Lämmle B (June 2001). "Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease".
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Sonneveld MA, de Maat MP, Portegies ML, Kavousi M, Hofman A, Turecek PL, Rottensteiner H, Scheiflinger F, Koudstaal PJ, Ikram MA, Leebeek FW (December 2015).
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domains. ADAMTS13 in fact has eight thrombospondin domains. It has no hydrophobic transmembrane domain, and hence it is not anchored in the cell membrane.
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Fujimura Y, Matsumoto M, Yagi H, Yoshioka A, Matsui T, Titani K (January 2002). "Von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome".
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Low levels of ADAMTS13 are also associated with an increased risk of arterial thrombosis, including myocardial infarction and cerebrovascular disease.
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Sonneveld MA, de Maat MP, Leebeek FW (July 2014). "Von Willebrand factor and ADAMTS13 in arterial thrombosis: a systematic review and meta-analysis".
1472: 2849: 1454: 3203: 1275:(see below), was characterized in its familial form by the presence in plasma of unusually large von Willebrand factor multimers (ULVWF). 2443: 2536: 2500: 2427: 1338: 1268: 1118: 305: 2902: 1272: 1125: 282: 2436: 1974:"Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura" 1663:
Maino A, Siegerink B, Lotta LA, Crawley JT, le Cessie S, Leebeek FW, Lane DA, Lowe GD, Peyvandi F, Rosendaal FR (August 2015).
1441: 1420: 2449: 2493: 3058: 1437: 204: 179: 52: 1416: 3173: 2854: 2240:"The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP)" 318: 217: 121: 311: 210: 1664: 2748: 2743: 1317:, all of which are characterised by a protease domain (the part that performs the protein hydrolysis), an adjacent 3043: 3159: 3146: 3133: 3120: 3107: 3094: 3081: 2527: 3053: 2180:
Kokame K, Miyata T (January 2004). "Genetic defects leading to hereditary thrombotic thrombocytopenic purpura".
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Plaimauer B, Scheiflinger F (January 2004). "Expression and characterization of recombinant human ADAMTS-13".
1375:), it has been accepted that increased exposure of vWf to ADAMTS13 due to various reasons would predispose to 2209:
Schneppenheim R, Budde U, Hassenpflug W, Obser T (January 2004). "Severe ADAMTS-13 deficiency in childhood".
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Denorme F, Kraft P, Pareyn I, Drechsler C, Deckmyn H, Vanhoorelbeke K, Kleinschnitz C, De Meyer SF (2017).
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LĂłpez JA, Dong JF (January 2004). "Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells".
1665:"Plasma ADAMTS-13 levels and the risk of myocardial infarction: an individual patient data meta-analysis" 2976: 2895: 2839: 1360: 1225: 112:, ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP, ADAM metallopeptidase with thrombospondin type 1 motif 13 3048: 2316:
George JN (May 2005). "ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome".
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Moake JL (January 2004). "von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura".
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Tsai HM (October 2002). "Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura".
108: 3012: 2455: 1372: 1101: 1097: 1093: 1089: 1034: 1030: 1026: 1022: 3208: 2945: 2368: 2304: 2081: 2038: 1960: 1923: 1882: 1705: 1534: 153: 1068: 1064: 1060: 1001: 997: 993: 989: 2485: 1554:"Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura" 556: 2462: 2398: 2360: 2325: 2296: 2261: 2226: 2197: 2168: 2139: 2110: 2073: 2030: 1995: 1952: 1915: 1874: 1845: 1805: 1746: 1697: 1645: 1610: 1575: 1526: 149: 101: 45: 2991: 2986: 2960: 2888: 2788: 2390: 2350: 2286: 2251: 2218: 2189: 2160: 2131: 2102: 2063: 2020: 1985: 1944: 1907: 1866: 1837: 1795: 1785: 1736: 1689: 1679: 1637: 1602: 1565: 1516: 1299: 398: 329: 273: 228: 2422: 28: 3213: 3038: 3022: 2935: 2544: 1218: 373: 2009:"Is severe deficiency of ADAMTS-13 specific for thrombotic thrombocytopenic purpura? Yes" 1553: 1781: 3187: 3076: 3017: 2222: 2193: 2164: 2135: 2106: 1990: 1973: 1895: 1800: 1765: 1570: 1379:
by causing increased degradation of vWf. This phenomenon is characterised by a form of
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Tang BL (January 2001). "ADAMTS: a novel family of extracellular matrix proteases".
1766:"Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease" 1538: 133: 2930: 1368: 391: 170: 2394: 2339:"Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions" 1709: 1349:
and characterisation of IgG inhibitors. Since the discovery of ADAMTS13, specific
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directed against this same enzyme caused TTP in a majority of non-familial cases.
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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
1318: 1229: 3182: 2291: 2274: 1725:"Low ADAMTS13 activity is associated with an increased risk of ischemic stroke" 1641: 1521: 1504: 474: 2834: 2706: 2052:"Is ADAMTS-13 deficiency specific for thrombotic thrombocytopenic purpura? No" 1948: 1376: 1342: 1283: 1256: 290: 187: 137: 3128: 3102: 1417:
ENSG00000281244 GRCh38: Ensembl release 89: ENSG00000160323, ENSG00000281244
815: 534: 412: 357: 344: 256: 243: 145: 2475:(A disintegrin and metalloproteinase with thrombospondin motifs 13) at the 2402: 2364: 2329: 2300: 2265: 2230: 2201: 2172: 2143: 2114: 2077: 2034: 1999: 1956: 1919: 1878: 1849: 1809: 1750: 1701: 1693: 1649: 1614: 1606: 1579: 1530: 1353:
on its surface have been shown to be the target of inhibitory antibodies.
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and degrades large vWf multimers, decreasing their activity, hence
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Genomically, ADAMTS13 shares many properties with the 19 member
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Overview of all the structural information available in the
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The International Journal of Biochemistry & Cell Biology
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in the small blood vessels. In addition, they reported that
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online database for peptidases and their inhibitors:
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Deficiency of ADAMTS13 was originally discovered in
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dentate gyrus of hippocampal formation granule cell
1978:Arteriosclerosis, Thrombosis, and Vascular Biology 1595:Best Practice & Research. Clinical Haematology 1433: 1431: 1429: 1412: 1410: 1408: 328: 227: 1278:In 1994, vWF was shown to be cleaved between a 2238:Kremer Hovinga JA, Studt JD, Lämmle B (2005). 1438:GRCm38: Ensembl release 89: ENSMUSG00000014852 2896: 2501: 2244:Pathophysiology of Haemostasis and Thrombosis 1558:Journal of the American Society of Nephrology 8: 2273:Levy GG, Motto DG, Ginsburg D (July 2005). 1503:Levy GG, Motto DG, Ginsburg D (July 2005). 2903: 2889: 2881: 2508: 2494: 2486: 1498: 1496: 1494: 1492: 1490: 1488: 1486: 811: 786:cellular response to tumor necrosis factor 581: 369: 268: 165: 63: 2354: 2290: 2255: 2067: 2024: 1989: 1799: 1789: 1740: 1683: 1569: 1520: 3178: 1404: 1345:form as well, owing to its response to 1211:von Willebrand factor-cleaving protease 771:cellular response to lipopolysaccharide 1898:(September 2002). "ADAMTS13 and TTP". 18: 2850:Pregnancy-associated plasma protein A 2343:Journal of Thrombosis and Haemostasis 2056:Journal of Thrombosis and Haemostasis 2013:Journal of Thrombosis and Haemostasis 1672:Journal of Thrombosis and Haemostasis 766:cellular response to interferon-gamma 333: 294: 289: 232: 191: 186: 7: 2428:Online Mendelian Inheritance in Man 1859:International Journal of Hematology 1339:thrombotic thrombocytopenic purpura 1269:thrombotic thrombocytopenic purpura 1240:acts to reduce thrombus formation. 1228:(vWf), a large protein involved in 741:integrin-mediated signaling pathway 2383:The American Journal of Cardiology 2223:10.1053/j.seminhematol.2003.10.007 2194:10.1053/j.seminhematol.2003.10.002 2165:10.1053/j.seminhematol.2003.10.006 2136:10.1053/j.seminhematol.2003.10.004 2107:10.1053/j.seminhematol.2003.10.003 1991:10.1161/01.ATV.0000058401.34021.D4 1571:10.1097/01.ASN.0000060805.04118.4C 1273:microangiopathic hemolytic anemias 1267:Since 1982 it had been known that 1079: 1050: 1012: 979: 955: 936: 910: 887: 861: 842: 781:cellular response to interleukin-4 551: 469: 407: 386: 14: 1337:, the recurring familial form of 1291:was associated with formation of 736:response to tumor necrosis factor 3181: 2356:10.1111/j.1538-7836.2005.01360.x 2069:10.1046/j.1538-7836.2003.00170.x 2026:10.1046/j.1538-7836.2003.00169.x 1912:10.1097/00062752-200209000-00001 1359:Finally, since the link between 555: 317: 310: 304: 281: 216: 209: 203: 178: 27: 701:glycoprotein metabolic process 566:More reference expression data 535:More reference expression data 427:right hemisphere of cerebellum 1: 2395:10.1016/j.amjcard.2007.03.095 1937:Journal of Molecular Medicine 1900:Current Opinion in Hematology 1842:10.1016/S1357-2725(00)00061-3 1367:was proven to be due to high 629:metalloendopeptidase activity 302: 201: 2855:Bone morphogenetic protein 1 1791:10.1371/journal.pone.0179258 1742:10.1182/blood-2015-05-643338 1204:pondin type 1 motif, member 711:response to interferon-gamma 3204:Genes on human chromosome 9 751:response to toxic substance 660:endoplasmic reticulum lumen 3230: 2454:gene details page in the 2318:Current Hematology Reports 2292:10.1182/blood-2004-10-4097 1642:10.1016/j.blre.2014.04.003 1522:10.1182/blood-2004-10-4097 1232:. It is secreted into the 3059:Michaelis–Menten kinetics 2682:Matrix metalloproteinases 2437:Secreted protein database 1949:10.1007/s00109-002-0369-8 1473:"Mouse PubMed Reference:" 1455:"Human PubMed Reference:" 1164: 1159: 1155: 1148: 1132: 1119:Chr 9: 133.41 – 133.46 Mb 1113: 1086: 1057: 1046: 1019: 986: 975: 962: 958: 943: 939: 930: 917: 913: 894: 890: 881: 868: 864: 849: 845: 836: 821: 814: 810: 794: 761:response to potassium ion 756:peptide catabolic process 706:response to interleukin-4 639:metallopeptidase activity 584: 580: 563: 554: 545: 532: 481: 472: 419: 410: 380: 372: 368: 351: 338: 301: 280: 271: 267: 250: 237: 200: 177: 168: 164: 119: 116: 106: 99: 94: 71: 66: 49: 44: 39: 35: 26: 21: 2951:Diffusion-limited enzyme 2865:Insulin-degrading enzyme 2050:Remuzzi G (April 2003). 1335:Upshaw Schulman Syndrome 1196:etalloproteinase with a 2337:Dong JF (August 2005). 1321:domain and one or more 1282:at position 1605 and a 2211:Seminars in Hematology 2182:Seminars in Hematology 2153:Seminars in Hematology 2124:Seminars in Hematology 2095:Seminars in Hematology 2007:Tsai HM (April 2003). 1972:Tsai HM (March 2003). 1607:10.1053/beha.2001.0142 1552:Tsai HM (April 2003). 1381:von Willebrand disease 1126:Chr 2: 26.86 – 26.9 Mb 604:endopeptidase activity 521:superior frontal gyrus 16:Metalloprotease enzyme 3044:Eadie–Hofstee diagram 2977:Allosteric regulation 2840:Procollagen peptidase 2521:metalloendopeptidases 1894:Zheng X, Majerus EM, 1361:aortic valve stenosis 1226:von Willebrand factor 513:primary visual cortex 447:primary visual cortex 3054:Lineweaver–Burk plot 2448:genome location and 746:cell-matrix adhesion 680:extracellular matrix 665:extracellular region 296:Chromosome 2 (mouse) 194:Chromosome 9 (human) 158:ADAMTS13 - orthologs 67:List of PDB id codes 40:Available structures 2456:UCSC Genome Browser 1782:2017PLoSO..1279258D 731:platelet activation 675:extracellular space 599:calcium ion binding 423:right lobe of liver 3013:Enzyme superfamily 2946:Enzyme promiscuity 2275:"ADAMTS13 turns 3" 1871:10.1007/BF02981975 1505:"ADAMTS13 turns 3" 1271:(TTP), one of the 1255:maps to the ninth 920:ENSMUSG00000014852 716:protein processing 689:Biological process 653:Cellular component 634:hydrolase activity 619:peptidase activity 592:Molecular function 509:hippocampus proper 435:right frontal lobe 431:right uterine tube 3169: 3168: 2878: 2877: 2257:10.1159/000083839 1685:10.1111/jth.13032 1175: 1174: 1171: 1170: 1144: 1143: 1109: 1108: 1076: 1075: 1042: 1041: 1009: 1008: 971: 970: 952: 951: 926: 925: 907: 906: 877: 876: 858: 857: 806: 805: 776:response to amine 721:blood coagulation 609:metal ion binding 576: 575: 572: 571: 541: 540: 528: 527: 466: 465: 364: 363: 263: 262: 90: 89: 86: 85: 50:Ortholog search: 3221: 3186: 3185: 3177: 3049:Hanes–Woolf plot 2992:Enzyme activator 2987:Enzyme inhibitor 2961:Enzyme catalysis 2905: 2898: 2891: 2882: 2545:Alpha secretases 2510: 2503: 2496: 2487: 2406: 2376: 2358: 2333: 2312: 2294: 2269: 2259: 2234: 2205: 2176: 2147: 2118: 2089: 2071: 2046: 2028: 2003: 1993: 1968: 1931: 1890: 1853: 1814: 1813: 1803: 1793: 1761: 1755: 1754: 1744: 1720: 1714: 1713: 1687: 1669: 1660: 1654: 1653: 1625: 1619: 1618: 1590: 1584: 1583: 1573: 1549: 1543: 1542: 1524: 1500: 1481: 1480: 1469: 1463: 1462: 1451: 1445: 1435: 1424: 1414: 1373:Heyde's syndrome 1209:)—also known as 1157: 1156: 1128: 1121: 1104: 1080: 1071: 1051: 1047:RefSeq (protein) 1037: 1013: 1004: 980: 956: 937: 911: 888: 862: 843: 812: 644:zinc ion binding 614:integrin binding 582: 568: 559: 552: 537: 477: 475:Top expressed in 470: 415: 413:Top expressed in 408: 387: 370: 360: 347: 336: 321: 314: 308: 297: 285: 269: 259: 246: 235: 220: 213: 207: 196: 182: 166: 160: 111: 104: 81: 64: 58: 37: 36: 31: 19: 3229: 3228: 3224: 3223: 3222: 3220: 3219: 3218: 3194: 3193: 3192: 3180: 3172: 3170: 3165: 3077:Oxidoreductases 3063: 3039:Enzyme kinetics 3027: 3023:List of enzymes 2996: 2965: 2936:Catalytic triad 2914: 2909: 2879: 2874: 2823: 2676: 2531: 2514: 2483: 2414: 2409: 2379: 2336: 2315: 2272: 2250:(5–6): 417–21. 2237: 2208: 2179: 2150: 2121: 2092: 2049: 2006: 1971: 1934: 1893: 1856: 1827: 1823: 1821:Further reading 1818: 1817: 1776:(6): e0179258. 1763: 1762: 1758: 1735:(25): 2739–46. 1722: 1721: 1717: 1678:(8): 1396–404. 1667: 1662: 1661: 1657: 1627: 1626: 1622: 1592: 1591: 1587: 1551: 1550: 1546: 1502: 1501: 1484: 1471: 1470: 1466: 1453: 1452: 1448: 1436: 1427: 1415: 1406: 1401: 1389: 1331: 1329:Role in disease 1311: 1265: 1246: 1219:metalloprotease 1166:View/Edit Mouse 1161:View/Edit Human 1124: 1117: 1114:Location (UCSC) 1100: 1096: 1092: 1088: 1067: 1063: 1059: 1033: 1029: 1025: 1021: 1000: 996: 992: 988: 901:ENSG00000281244 899: 897:ENSG00000160323 790: 684: 648: 624:protein binding 564: 533: 524: 519: 515: 511: 507: 503: 499: 495: 491: 487: 473: 462: 459:Brodmann area 9 457: 453: 451:pituitary gland 449: 445: 441: 437: 433: 429: 425: 411: 355: 342: 334: 324: 323: 322: 315: 295: 272:Gene location ( 254: 241: 233: 223: 222: 221: 214: 192: 169:Gene location ( 120: 107: 100: 73: 51: 17: 12: 11: 5: 3227: 3225: 3217: 3216: 3211: 3206: 3196: 3195: 3191: 3190: 3167: 3166: 3164: 3163: 3150: 3137: 3124: 3111: 3098: 3085: 3071: 3069: 3065: 3064: 3062: 3061: 3056: 3051: 3046: 3041: 3035: 3033: 3029: 3028: 3026: 3025: 3020: 3015: 3010: 3004: 3002: 3001:Classification 2998: 2997: 2995: 2994: 2989: 2984: 2979: 2973: 2971: 2967: 2966: 2964: 2963: 2958: 2953: 2948: 2943: 2938: 2933: 2928: 2922: 2920: 2916: 2915: 2910: 2908: 2907: 2900: 2893: 2885: 2876: 2875: 2873: 2872: 2867: 2862: 2857: 2852: 2847: 2842: 2837: 2831: 2829: 2825: 2824: 2822: 2821: 2816: 2811: 2806: 2801: 2796: 2791: 2786: 2781: 2776: 2771: 2766: 2761: 2756: 2751: 2746: 2741: 2736: 2731: 2726: 2721: 2720: 2719: 2714: 2704: 2703: 2702: 2697: 2686: 2684: 2678: 2677: 2675: 2674: 2669: 2664: 2659: 2654: 2649: 2644: 2639: 2634: 2629: 2624: 2619: 2614: 2609: 2604: 2599: 2594: 2589: 2584: 2579: 2574: 2569: 2568: 2567: 2562: 2557: 2552: 2541: 2539: 2533: 2532: 2515: 2513: 2512: 2505: 2498: 2490: 2481: 2480: 2459: 2440: 2434: 2425: 2413: 2412:External links 2410: 2408: 2407: 2377: 2334: 2313: 2270: 2235: 2206: 2177: 2148: 2119: 2090: 2047: 2004: 1969: 1943:(10): 639–47. 1932: 1891: 1854: 1824: 1822: 1819: 1816: 1815: 1756: 1715: 1655: 1620: 1585: 1564:(4): 1072–81. 1544: 1482: 1464: 1446: 1425: 1403: 1402: 1400: 1397: 1396: 1395: 1388: 1385: 1365:angiodysplasia 1347:plasmapheresis 1330: 1327: 1323:thrombospondin 1310: 1307: 1264: 1261: 1245: 1242: 1230:blood clotting 1173: 1172: 1169: 1168: 1163: 1153: 1152: 1146: 1145: 1142: 1141: 1139: 1137: 1130: 1129: 1122: 1115: 1111: 1110: 1107: 1106: 1084: 1083: 1077: 1074: 1073: 1055: 1054: 1048: 1044: 1043: 1040: 1039: 1017: 1016: 1010: 1007: 1006: 984: 983: 977: 973: 972: 969: 968: 960: 959: 953: 950: 949: 941: 940: 934: 928: 927: 924: 923: 915: 914: 908: 905: 904: 892: 891: 885: 879: 878: 875: 874: 866: 865: 859: 856: 855: 847: 846: 840: 834: 833: 828: 823: 819: 818: 808: 807: 804: 803: 792: 791: 789: 788: 783: 778: 773: 768: 763: 758: 753: 748: 743: 738: 733: 728: 723: 718: 713: 708: 703: 698: 692: 690: 686: 685: 683: 682: 677: 672: 667: 662: 656: 654: 650: 649: 647: 646: 641: 636: 631: 626: 621: 616: 611: 606: 601: 595: 593: 589: 588: 578: 577: 574: 573: 570: 569: 561: 560: 549: 543: 542: 539: 538: 530: 529: 526: 525: 523: 522: 518: 514: 510: 506: 502: 498: 494: 490: 486: 482: 479: 478: 467: 464: 463: 461: 460: 456: 452: 448: 444: 440: 436: 432: 428: 424: 420: 417: 416: 404: 403: 395: 384: 378: 377: 374:RNA expression 366: 365: 362: 361: 353: 349: 348: 340: 337: 332: 326: 325: 316: 309: 303: 299: 298: 293: 287: 286: 278: 277: 265: 264: 261: 260: 252: 248: 247: 239: 236: 231: 225: 224: 215: 208: 202: 198: 197: 190: 184: 183: 175: 174: 162: 161: 118: 114: 113: 105: 97: 96: 92: 91: 88: 87: 84: 83: 69: 68: 60: 59: 48: 42: 41: 33: 32: 24: 23: 15: 13: 10: 9: 6: 4: 3: 2: 3226: 3215: 3212: 3210: 3207: 3205: 3202: 3201: 3199: 3189: 3184: 3179: 3175: 3161: 3157: 3156: 3151: 3148: 3144: 3143: 3138: 3135: 3131: 3130: 3125: 3122: 3118: 3117: 3112: 3109: 3105: 3104: 3099: 3096: 3092: 3091: 3086: 3083: 3079: 3078: 3073: 3072: 3070: 3066: 3060: 3057: 3055: 3052: 3050: 3047: 3045: 3042: 3040: 3037: 3036: 3034: 3030: 3024: 3021: 3019: 3018:Enzyme family 3016: 3014: 3011: 3009: 3006: 3005: 3003: 2999: 2993: 2990: 2988: 2985: 2983: 2982:Cooperativity 2980: 2978: 2975: 2974: 2972: 2968: 2962: 2959: 2957: 2954: 2952: 2949: 2947: 2944: 2942: 2941:Oxyanion hole 2939: 2937: 2934: 2932: 2929: 2927: 2924: 2923: 2921: 2917: 2913: 2906: 2901: 2899: 2894: 2892: 2887: 2886: 2883: 2871: 2868: 2866: 2863: 2861: 2858: 2856: 2853: 2851: 2848: 2846: 2843: 2841: 2838: 2836: 2833: 2832: 2830: 2826: 2820: 2817: 2815: 2812: 2810: 2807: 2805: 2802: 2800: 2797: 2795: 2792: 2790: 2787: 2785: 2782: 2780: 2777: 2775: 2772: 2770: 2767: 2765: 2762: 2760: 2757: 2755: 2752: 2750: 2747: 2745: 2742: 2740: 2737: 2735: 2732: 2730: 2727: 2725: 2722: 2718: 2715: 2713: 2710: 2709: 2708: 2705: 2701: 2698: 2696: 2693: 2692: 2691: 2688: 2687: 2685: 2683: 2679: 2673: 2670: 2668: 2665: 2663: 2660: 2658: 2655: 2653: 2650: 2648: 2645: 2643: 2640: 2638: 2635: 2633: 2630: 2628: 2625: 2623: 2620: 2618: 2615: 2613: 2610: 2608: 2605: 2603: 2600: 2598: 2595: 2593: 2590: 2588: 2585: 2583: 2580: 2578: 2575: 2573: 2570: 2566: 2563: 2561: 2558: 2556: 2553: 2551: 2548: 2547: 2546: 2543: 2542: 2540: 2538: 2537:ADAM proteins 2534: 2529: 2526: 2522: 2518: 2511: 2506: 2504: 2499: 2497: 2492: 2491: 2488: 2484: 2478: 2474: 2473: 2468: 2464: 2460: 2457: 2453: 2452: 2447: 2446: 2441: 2438: 2435: 2433: 2429: 2426: 2424: 2420: 2416: 2415: 2411: 2404: 2400: 2396: 2392: 2389:(5): 758–63. 2388: 2384: 2381:infarction". 2378: 2374: 2370: 2366: 2362: 2357: 2352: 2349:(8): 1710–6. 2348: 2344: 2340: 2335: 2331: 2327: 2323: 2319: 2314: 2310: 2306: 2302: 2298: 2293: 2288: 2284: 2280: 2276: 2271: 2267: 2263: 2258: 2253: 2249: 2245: 2241: 2236: 2232: 2228: 2224: 2220: 2216: 2212: 2207: 2203: 2199: 2195: 2191: 2187: 2183: 2178: 2174: 2170: 2166: 2162: 2158: 2154: 2149: 2145: 2141: 2137: 2133: 2129: 2125: 2120: 2116: 2112: 2108: 2104: 2100: 2096: 2091: 2087: 2083: 2079: 2075: 2070: 2065: 2061: 2057: 2053: 2048: 2044: 2040: 2036: 2032: 2027: 2022: 2019:(4): 625–31. 2018: 2014: 2010: 2005: 2001: 1997: 1992: 1987: 1984:(3): 388–96. 1983: 1979: 1975: 1970: 1966: 1962: 1958: 1954: 1950: 1946: 1942: 1938: 1933: 1929: 1925: 1921: 1917: 1913: 1909: 1906:(5): 389–94. 1905: 1901: 1897: 1892: 1888: 1884: 1880: 1876: 1872: 1868: 1864: 1860: 1855: 1851: 1847: 1843: 1839: 1835: 1831: 1826: 1825: 1820: 1811: 1807: 1802: 1797: 1792: 1787: 1783: 1779: 1775: 1771: 1767: 1760: 1757: 1752: 1748: 1743: 1738: 1734: 1730: 1726: 1719: 1716: 1711: 1707: 1703: 1699: 1695: 1694:10044/1/26935 1691: 1686: 1681: 1677: 1673: 1666: 1659: 1656: 1651: 1647: 1643: 1639: 1636:(4): 167–78. 1635: 1631: 1630:Blood Reviews 1624: 1621: 1616: 1612: 1608: 1604: 1601:(2): 437–54. 1600: 1596: 1589: 1586: 1581: 1577: 1572: 1567: 1563: 1559: 1555: 1548: 1545: 1540: 1536: 1532: 1528: 1523: 1518: 1514: 1510: 1506: 1499: 1497: 1495: 1493: 1491: 1489: 1487: 1483: 1478: 1474: 1468: 1465: 1460: 1456: 1450: 1447: 1443: 1439: 1434: 1432: 1430: 1426: 1422: 1418: 1413: 1411: 1409: 1405: 1398: 1394: 1391: 1390: 1386: 1384: 1382: 1378: 1374: 1370: 1366: 1362: 1357: 1354: 1352: 1348: 1344: 1340: 1336: 1328: 1326: 1324: 1320: 1316: 1315:ADAMTS family 1308: 1306: 1304: 1301: 1297: 1294: 1290: 1285: 1281: 1276: 1274: 1270: 1262: 1260: 1258: 1254: 1251: 1243: 1241: 1239: 1235: 1231: 1227: 1224:that cleaves 1223: 1220: 1216: 1213:(VWFCP)—is a 1212: 1208: 1207: 1203: 1199: 1195: 1191: 1187: 1184: 1179: 1167: 1162: 1158: 1154: 1151: 1147: 1140: 1138: 1135: 1131: 1127: 1123: 1120: 1116: 1112: 1105: 1103: 1099: 1095: 1091: 1085: 1081: 1078: 1072: 1070: 1066: 1062: 1056: 1052: 1049: 1045: 1038: 1036: 1032: 1028: 1024: 1018: 1014: 1011: 1005: 1003: 999: 995: 991: 985: 981: 978: 976:RefSeq (mRNA) 974: 967: 966: 961: 957: 954: 948: 947: 942: 938: 935: 933: 929: 922: 921: 916: 912: 909: 903: 902: 898: 893: 889: 886: 884: 880: 873: 872: 867: 863: 860: 854: 853: 848: 844: 841: 839: 835: 832: 829: 827: 824: 820: 817: 813: 809: 802: 798: 793: 787: 784: 782: 779: 777: 774: 772: 769: 767: 764: 762: 759: 757: 754: 752: 749: 747: 744: 742: 739: 737: 734: 732: 729: 727: 724: 722: 719: 717: 714: 712: 709: 707: 704: 702: 699: 697: 694: 693: 691: 688: 687: 681: 678: 676: 673: 671: 668: 666: 663: 661: 658: 657: 655: 652: 651: 645: 642: 640: 637: 635: 632: 630: 627: 625: 622: 620: 617: 615: 612: 610: 607: 605: 602: 600: 597: 596: 594: 591: 590: 587: 586:Gene ontology 583: 579: 567: 562: 558: 553: 550: 548: 544: 536: 531: 520: 516: 512: 508: 504: 500: 496: 492: 488: 484: 483: 480: 476: 471: 468: 458: 455:apex of heart 454: 450: 446: 442: 438: 434: 430: 426: 422: 421: 418: 414: 409: 406: 405: 402: 400: 396: 394: 393: 389: 388: 385: 383: 379: 375: 371: 367: 359: 354: 350: 346: 341: 331: 327: 320: 313: 307: 300: 292: 288: 284: 279: 275: 270: 266: 258: 253: 249: 245: 240: 230: 226: 219: 212: 206: 199: 195: 189: 185: 181: 176: 172: 167: 163: 159: 155: 151: 147: 143: 139: 135: 131: 127: 123: 115: 110: 103: 98: 93: 82: 80: 76: 70: 65: 62: 61: 57: 54: 47: 43: 38: 34: 30: 25: 20: 3155:Translocases 3152: 3139: 3126: 3113: 3100: 3090:Transferases 3087: 3074: 2931:Binding site 2690:Collagenases 2671: 2482: 2470: 2450: 2444: 2386: 2382: 2346: 2342: 2324:(3): 167–9. 2321: 2317: 2282: 2278: 2247: 2243: 2214: 2210: 2188:(1): 34–40. 2185: 2181: 2159:(1): 24–33. 2156: 2152: 2130:(1): 15–23. 2127: 2123: 2098: 2094: 2062:(4): 632–4. 2059: 2055: 2016: 2012: 1981: 1977: 1940: 1936: 1903: 1899: 1865:(1): 25–34. 1862: 1858: 1836:(1): 33–44. 1833: 1829: 1773: 1769: 1759: 1732: 1728: 1718: 1675: 1671: 1658: 1633: 1629: 1623: 1598: 1594: 1588: 1561: 1557: 1547: 1512: 1508: 1476: 1467: 1458: 1449: 1369:shear stress 1358: 1355: 1332: 1312: 1296:microthrombi 1277: 1266: 1249: 1247: 1237: 1217:-containing 1210: 1205: 1201: 1197: 1193: 1189: 1185: 1182: 1181: 1177: 1176: 1102:NP_001277394 1098:NP_001277393 1094:NP_001277392 1090:NP_001001322 1087: 1058: 1035:NM_001290465 1031:NM_001290464 1027:NM_001290463 1023:NM_001001322 1020: 987: 963: 944: 918: 895: 869: 850: 830: 825: 670:cell surface 439:right testis 397: 390: 255:133,459,402 242:133,414,358 117:External IDs 72: 2926:Active site 2860:Lysostaphin 2845:Thermolysin 2707:Gelatinases 2285:(1): 11–7. 2217:(1): 83–9. 2101:(1): 4–14. 1515:(1): 11–7. 1383:(type 2a). 1319:disintegrin 1188:isintegrin 726:proteolysis 497:bone marrow 443:left testis 356:26,899,640 343:26,863,428 335:2|2 A3 95:Identifiers 3198:Categories 3129:Isomerases 3103:Hydrolases 2970:Regulation 2835:Neprilysin 1444:, May 2017 1423:, May 2017 1399:References 1343:autoimmune 1309:Proteomics 1303:antibodies 1284:methionine 1257:chromosome 696:hemostasis 401:(ortholog) 138:HomoloGene 3209:EC 3.4.24 3008:EC number 2517:Proteases 1896:Sadler JE 1263:Discovery 1069:NP_620596 1065:NP_620595 1061:NP_620594 1002:NM_139028 998:NM_139027 994:NM_139026 990:NM_139025 816:Orthologs 505:esophagus 146:GeneCards 3032:Kinetics 2956:Cofactor 2919:Activity 2870:ZMPSTE24 2672:ADAMTS13 2667:ADAMTS12 2662:ADAMTS10 2451:ADAMTS13 2445:ADAMTS13 2430:(OMIM): 2403:17719316 2373:33464866 2365:16102037 2330:15865866 2309:25645477 2301:15774620 2266:15692254 2231:14727263 2202:14727257 2173:14727256 2144:14727255 2115:14727254 2086:10742104 2078:12871391 2043:26485194 2035:12871390 2000:12615692 1965:24992893 1957:12395148 1928:19457473 1920:12172456 1887:19926816 1879:11843286 1850:11167130 1810:28591212 1770:PLOS ONE 1751:26511134 1702:26073931 1650:24825749 1615:11686108 1580:12660343 1539:25645477 1531:15774620 1440:– 1419:– 1387:See also 1377:bleeding 1351:epitopes 1293:platelet 1289:protease 1280:tyrosine 1259:(9q34). 1250:ADAMTS13 1244:Genetics 1238:ADAMTS13 1178:ADAMTS13 1150:Wikidata 795:Sources: 517:yolk sac 150:ADAMTS13 109:ADAMTS13 22:ADAMTS13 3188:Biology 3142:Ligases 2912:Enzymes 2657:ADAMTS9 2652:ADAMTS8 2647:ADAMTS5 2642:ADAMTS4 2637:ADAMTS3 2632:ADAMTS2 2627:ADAMTS1 2477:PDBe-KB 2467:UniProt 2423:M12.241 1801:5462472 1778:Bibcode 1442:Ensembl 1421:Ensembl 1393:ADAMTS5 932:UniProt 883:Ensembl 822:Species 801:QuickGO 376:pattern 134:2685556 102:Aliases 3214:ADAMTS 3174:Portal 3116:Lyases 2794:MMP23B 2789:MMP23A 2622:ADAM33 2617:ADAM28 2612:ADAM23 2607:ADAM22 2602:ADAM18 2597:ADAM15 2592:ADAM12 2587:ADAM11 2565:ADAM19 2560:ADAM17 2555:ADAM10 2528:3.4.24 2472:Q76LX8 2442:Human 2432:274150 2419:MEROPS 2401:  2371:  2363:  2328:  2307:  2299:  2264:  2229:  2200:  2171:  2142:  2113:  2084:  2076:  2041:  2033:  1998:  1963:  1955:  1926:  1918:  1885:  1877:  1848:  1808:  1798:  1749:  1710:324472 1708:  1700:  1648:  1613:  1578:  1537:  1529:  1222:enzyme 1200:hrombo 1136:search 1134:PubMed 965:Q769J6 946:Q76LX8 871:279028 838:Entrez 547:BioGPS 501:embryo 493:morula 234:9q34.2 126:604134 3068:Types 2828:Other 2819:MMP28 2814:MMP27 2809:MMP26 2804:MMP25 2799:MMP24 2784:MMP21 2779:MMP20 2774:MMP19 2769:MMP17 2764:MMP16 2759:MMP15 2754:MMP14 2749:MMP13 2744:MMP12 2739:MMP11 2734:MMP10 2582:ADAM8 2577:ADAM7 2572:ADAM2 2550:ADAM9 2439:entry 2369:S2CID 2305:S2CID 2279:Blood 2082:S2CID 2039:S2CID 1961:S2CID 1924:S2CID 1883:S2CID 1729:Blood 1706:S2CID 1668:(PDF) 1535:S2CID 1509:Blood 1234:blood 852:11093 831:Mouse 826:Human 797:Amigo 485:liver 399:Mouse 392:Human 339:Start 274:Mouse 238:Start 171:Human 142:16372 3160:list 3153:EC7 3147:list 3140:EC6 3134:list 3127:EC5 3121:list 3114:EC4 3108:list 3101:EC3 3095:list 3088:EC2 3082:list 3075:EC1 2729:MMP7 2724:MMP3 2717:MMP9 2712:MMP2 2700:MMP8 2695:MMP1 2465:for 2417:The 2399:PMID 2361:PMID 2326:PMID 2297:PMID 2262:PMID 2227:PMID 2198:PMID 2169:PMID 2140:PMID 2111:PMID 2074:PMID 2031:PMID 1996:PMID 1953:PMID 1916:PMID 1875:PMID 1846:PMID 1806:PMID 1747:PMID 1698:PMID 1646:PMID 1611:PMID 1576:PMID 1527:PMID 1363:and 1253:gene 1248:The 1215:zinc 382:Bgee 330:Band 291:Chr. 229:Band 188:Chr. 122:OMIM 79:3GHN 75:3GHM 56:RCSB 53:PDBe 2463:PDB 2391:doi 2387:100 2351:doi 2287:doi 2283:106 2252:doi 2219:doi 2190:doi 2161:doi 2132:doi 2103:doi 2064:doi 2021:doi 1986:doi 1945:doi 1908:doi 1867:doi 1838:doi 1796:PMC 1786:doi 1737:doi 1733:126 1690:hdl 1680:doi 1638:doi 1603:doi 1566:doi 1517:doi 1513:106 1300:IgG 1192:nd 352:End 251:End 154:OMA 130:MGI 46:PDB 3200:: 2525:EC 2519:: 2469:: 2397:. 2385:. 2367:. 2359:. 2345:. 2341:. 2320:. 2303:. 2295:. 2281:. 2277:. 2260:. 2248:33 2246:. 2242:. 2225:. 2215:41 2213:. 2196:. 2186:41 2184:. 2167:. 2157:41 2155:. 2138:. 2128:41 2126:. 2109:. 2099:41 2097:. 2080:. 2072:. 2058:. 2054:. 2037:. 2029:. 2015:. 2011:. 1994:. 1982:23 1980:. 1976:. 1959:. 1951:. 1941:80 1939:. 1922:. 1914:. 1902:. 1881:. 1873:. 1863:75 1861:. 1844:. 1834:33 1832:. 1804:. 1794:. 1784:. 1774:12 1772:. 1768:. 1745:. 1731:. 1727:. 1704:. 1696:. 1688:. 1676:13 1674:. 1670:. 1644:. 1634:28 1632:. 1609:. 1599:14 1597:. 1574:. 1562:14 1560:. 1556:. 1533:. 1525:. 1511:. 1507:. 1485:^ 1475:. 1457:. 1428:^ 1407:^ 1206:13 799:/ 358:bp 345:bp 257:bp 244:bp 152:; 148:: 144:; 140:: 136:; 132:: 128:; 124:: 77:, 3176:: 3162:) 3158:( 3149:) 3145:( 3136:) 3132:( 3123:) 3119:( 3110:) 3106:( 3097:) 3093:( 3084:) 3080:( 2904:e 2897:t 2890:v 2530:) 2523:( 2509:e 2502:t 2495:v 2479:. 2458:. 2405:. 2393:: 2375:. 2353:: 2347:3 2332:. 2322:4 2311:. 2289:: 2268:. 2254:: 2233:. 2221:: 2204:. 2192:: 2175:. 2163:: 2146:. 2134:: 2117:. 2105:: 2088:. 2066:: 2060:1 2045:. 2023:: 2017:1 2002:. 1988:: 1967:. 1947:: 1930:. 1910:: 1904:9 1889:. 1869:: 1852:. 1840:: 1812:. 1788:: 1780:: 1753:. 1739:: 1712:. 1692:: 1682:: 1652:. 1640:: 1617:. 1605:: 1582:. 1568:: 1541:. 1519:: 1479:. 1461:. 1371:( 1202:s 1198:t 1194:m 1190:a 1186:d 1183:a 1180:( 276:) 173:) 156::

Index


PDB
PDBe
RCSB
3GHM
3GHN
Aliases
ADAMTS13
OMIM
604134
MGI
2685556
HomoloGene
16372
GeneCards
ADAMTS13
OMA
ADAMTS13 - orthologs
Human
Chromosome 9 (human)
Chr.
Chromosome 9 (human)
Chromosome 9 (human)
Genomic location for ADAMTS13
Genomic location for ADAMTS13
Band
bp
bp
Mouse
Chromosome 2 (mouse)

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