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C1-inhibitor

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315: 1192:. Also, C4 and C2 cleavage goes unchecked, resulting in auto-activation of the complement system. In its most common form, it presents as marked swelling of the face, mouth and/or airway that occurs spontaneously or to minimal triggers (such as mild trauma), but such swelling can occur in any part of the body. In 85% of the cases, the levels of C1-inhibitor are low, while in 15% the protein circulates in normal amounts but it is dysfunctional. In addition to the episodes of facial swelling and/or abdominal pain, it also predisposes to 292: 189: 214: 566: 1581:(the most commonly used organism for this purpose) lacks the eukaryotic ability to glycosylate proteins; as C1-inhibitor is particularly heavily glycosylated, this sialylated recombinant form would have a short circulatory life (the carbohydrates are not relevant to the inhibitor function). Therefore, C1-inhibitor has also been produced in glycosylated form using transgenic rabbits. This form of recombinant C1-inhibitor also has been given 321: 220: 3567: 1553:, a pharmaceutical-grade C1-inhibitor, was approved for the use of HAE in 2008 in the US after having been available in Europe for decades. It is a highly purified, pasteurized and nanofiltered plasma-derived C1 esterase inhibitor product; it has been approved for routine prophylaxis against angioedema attacks in adolescent and adult patients with HAE. 4357: 68: 42: 1188:("hereditary angioneurotic edema"), or swelling due to leakage of fluid from blood vessels into connective tissue. Deficiency of C1-inhibitor permits plasma kallikrein activation, which leads to the production of the vasoactive peptide 2191: 2507:
Caliezi C, Wuillemin WA, Zeerleder S, Redondo M, Eisele B, Hack CE (March 2000). "C1-Esterase inhibitor: an anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema".
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Tosi M, Duponchel C, Bourgarel P, Colomb M, Meo T (1986). "Molecular cloning of human C1 inhibitor: sequence homologies with alpha 1-antitrypsin and other members of the serpins superfamily".
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Carter PE, Duponchel C, Tosi M, Fothergill JE (April 1991). "Complete nucleotide sequence of the gene for human C1 inhibitor with an unusually high density of Alu elements".
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Davis AE, Aulak K, Parad RB, Stecklein HP, Eldering E, Hack CE, et al. (August 1992). "C1 inhibitor hinge region mutations produce dysfunction by different mechanisms".
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Carter PE, Duponchel C, Tosi M, Fothergill JE (April 1991). "Complete nucleotide sequence of the gene for human C1 inhibitor with an unusually high density of Alu elements".
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A recombinant C1-inhibitor obtained from the milk of transgenic rabbits, conestat alfa (brand name Ruconest), is approved for the treatment of acute HAE attacks in adults.
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The activation of the complement cascade can cause damage to cells, therefore the inhibition of the complement cascade can work as a medicine in certain conditions.
1571:. Risks of infectious disease transmission (viruses, prions, etc.) and relative expense of isolation prevented widespread use. It is also possible to produce it by 1247: 2792:
Siddique Z, McPhaden AR, Lappin DF, Whaley K (December 1991). "An RNA splice site mutation in the C1-inhibitor gene causes type I hereditary angio-oedema".
3940: 3099:"Identification of a new P1 residue mutation (444Arg----Ser) in a dysfunctional C1 inhibitor protein contained in a type II hereditary angioedema plasma" 834: 815: 150: 3321: 2640:
Siddique Z, McPhaden AR, McCluskey D, Whaley K (1992). "A single base deletion from the C1-inhibitor gene causes type I hereditary angio-oedema".
2257: 1739: 3009:"Type II hereditary angioneurotic edema that may result from a single nucleotide change in the codon for alanine-436 in the C1 inhibitor gene" 4377: 3546: 1670: 3675: 1652: 3255: 3060:
Theriault A, Whaley K, McPhaden AR, Boyd E, Connor JM (April 1990). "Regional assignment of the human C1-inhibitor gene to 11q11-q13.1".
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Theriault A, Whaley K, McPhaden AR, Boyd E, Connor JM (April 1990). "Regional assignment of the human C1-inhibitor gene to 11q11-q13.1".
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Davis AE, Bissler JJ, Cicardi M (December 1993). "Mutations in the C1 inhibitor gene that result in hereditary angioneurotic edema".
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Bernstein JA (January 2008). "Hereditary angioedema: a current state-of-the-art review, VIII: current status of emerging therapies".
3844: 3786: 3724: 3670: 3665: 3607: 2333:"Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance" 1049: 291: 1152:) is not essential for C1-inhibitor to inhibit proteases. This domain has no similarity to other proteins. C1-inhibitor is highly 2384:"Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema" 3933: 1912:
Davis AE (January 2008). "Hereditary angioedema: a current state-of-the-art review, III: mechanisms of hereditary angioedema".
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Cicardi M, Zingale L, Zanichelli A, Pappalardo E, Cicardi B (November 2005). "C1 inhibitor: molecular and clinical aspects".
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serpin domain is similar to other serpins, which is the part of C1-inhibitor that provides the inhibitory activity. The
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Stein PE, Carrell RW (February 1995). "What do dysfunctional serpins tell us about molecular mobility and disease?".
320: 219: 2831:"Nonsense mutations affect C1 inhibitor messenger RNA levels in patients with type I hereditary angioneurotic edema" 2284:"Training patients for self-administration of a new subcutaneous C1-inhibitor concentrate for hereditary angioedema" 1549:
Blood-derived C1-inhibitor is effective but does carry the risk associated with the use of any human blood product.
4382: 3926: 3885: 3487: 1200:, due to its consumptive effect on complement factors 3 and 4. Mutations in the gene that codes for C1-inhibitor, 4209: 3539: 3692: 3630: 3482: 3472: 879: 138: 4289: 4159: 4124: 4119: 4014: 4009: 4004: 1949:"Association between the SERPING1 gene and age-related macular degeneration: a two-stage case-control study" 1315: 860: 4179: 4169: 4164: 4114: 4093: 4038: 4021: 2956:"Clusters of intragenic Alu repeats predispose the human C1 inhibitor locus to deleterious rearrangements" 1559:
Other products also have been introduced including plasma-derived products such as Berinert and Haegarda.
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Koles K, van Berkel PH, Pieper FR, Nuijens JH, Mannesse ML, Vliegenthart JF, et al. (January 2004).
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Lappin D, Whaley K (July 1989). "Regulation of C1-inhibitor synthesis by interferons and other agents".
1733: 1185: 202: 2903:"Dysfunctional C1 inhibitor Ta: deletion of Lys-251 results in acquisition of an N-glycosylation site" 1585:
status for delayed graft function following organ transplantation and for capillary leakage syndrome.
4271: 4266: 3532: 3020: 2967: 2914: 2009: 1205: 117: 4248: 4228: 4218: 4067: 3781: 3682: 3660: 3655: 3640: 3635: 3454: 3267: 1435: 1260: 1197: 1004: 949: 3367: 3128: 3085: 2817: 2737: 2694: 2582: 2413: 1859: 1816: 1193: 162: 2433:"N- and O-glycans of recombinant human C1 inhibitor expressed in the milk of transgenic rabbits" 2081: 1136:
superfamily of proteins. It can be noted that, unlike most family members, C1-inhibitor has a 2-
1025: 1000: 974: 953: 3177:"Type I C1 inhibitor deficiency with a small messenger RNA resulting from deletion of one exon" 565: 3949: 3286: 3235: 3206: 3163: 3120: 3077: 3048: 2995: 2942: 2889: 2860: 2809: 2780: 2729: 2686: 2657: 2628: 2599: 2574: 2545: 2517: 2489: 2454: 2405: 2364: 2313: 2035: 1978: 1929: 1894: 1851: 1808: 1770: 1721: 1408: 1395: 1383: 1229: 1121: 158: 110: 58: 2708:"A dysfunctional C1 inhibitor protein with a new reactive center mutation (Arg-444-->Leu)" 2165: 1444: 4323: 4276: 4098: 3593: 3459: 3227: 3196: 3188: 3153: 3110: 3069: 3038: 3028: 2985: 2975: 2932: 2922: 2881: 2850: 2842: 2801: 2770: 2762: 2719: 2678: 2649: 2620: 2566: 2481: 2444: 2395: 2354: 2344: 2303: 2295: 2025: 2017: 1968: 1960: 1921: 1886: 1843: 1800: 1762: 1711: 1701: 1577: 407: 338: 282: 237: 1505: 1498: 1451: 4361: 4052: 4048: 3890: 1572: 382: 1124:
to prevent spontaneous activation but also as the major regulator of the contact system.
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Murphy E, Donahue C, Omert L, Persons S, Tyma TJ, Chiao J, et al. (January 2019).
2030: 1997: 1973: 1948: 1890: 1716: 1689: 1568: 1156:, bearing both N- and O-glycans. N-terminal domain is especially heavily glycosylated. 1137: 3201: 3176: 3158: 3141: 2855: 2830: 2485: 1964: 1947:
Ennis S, Jomary C, Mullins R, Cree A, Chen X, Macleod A, et al. (November 2008).
1925: 749: 744: 739: 734: 729: 724: 719: 714: 709: 704: 699: 694: 689: 684: 679: 674: 658: 653: 648: 643: 638: 633: 617: 612: 607: 4371: 4138: 4133: 3477: 3428: 3387: 3382: 3295: 3231: 3115: 3098: 3043: 3008: 2990: 2955: 2937: 2902: 2724: 2707: 2136: 2110: 2058: 1347: 1153: 594: 17: 3132: 3089: 2821: 2741: 2698: 2586: 2417: 1863: 1820: 4334: 4329: 4317: 4299: 4294: 3776: 3645: 3463: 3418: 3409: 3372: 3362: 3348: 3338: 3306: 3278: 3273: 1352: 1342: 1337: 400: 179: 142: 2751:"Effect of interferon-gamma on complement gene expression in different cell types" 1567:
C1-inhibitor is contained in the human blood; it can, therefore, be isolated from
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Skriver K, Radziejewska E, Silbermann JA, Donaldson VH, Bock SC (February 1989).
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Law RH, Zhang Q, McGowan S, Buckle AM, Silverman GA, Wong W, et al. (2006).
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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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Proceedings of the National Academy of Sciences of the United States of America
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Proceedings of the National Academy of Sciences of the United States of America
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Proceedings of the National Academy of Sciences of the United States of America
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Frangi D, Cicardi M, Sica A, Colotta F, Agostoni A, Davis AE (September 1991).
2624: 2224: 2021: 1274: 483: 4174: 3999: 2400: 2383: 1804: 1189: 1173: 1145: 1141: 299: 196: 146: 4077: 3875: 3769: 3764: 3497: 3492: 3438: 3357: 2927: 2449: 2432: 1706: 1251: 1217: 1208:. At least 97 disease-causing mutations in this gene have been discovered. 779: 543: 421: 366: 353: 265: 252: 154: 2980: 2632: 2521: 2493: 2458: 2409: 2368: 2317: 2039: 1982: 1933: 1812: 1774: 1725: 3239: 3210: 3167: 3124: 3081: 3052: 3033: 2999: 2946: 2893: 2864: 2813: 2784: 2733: 2690: 2661: 2603: 2578: 2549: 1898: 1855: 1089: 1084: 41: 4238: 4072: 3821: 3816: 3801: 3796: 3791: 3759: 3754: 3749: 3744: 3734: 3729: 3719: 3707: 3702: 3697: 3687: 3175:
Ariga T, Igarashi T, Ramesh N, Parad R, Cicardi M, Davis AE (June 1989).
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Davis AE (January 2005). "The pathophysiology of hereditary angioedema".
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Levy NJ, Ramesh N, Cicardi M, Harrison RA, Davis AE (January 1990).
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Parad RB, Kramer J, Strunk RC, Rosen FS, Davis AE (September 1990).
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Frangi D, Aulak KS, Cicardi M, Harrison RA, Davis AE (April 1992).
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Davis AE (September 2004). "Biological effects of C1 inhibitor".
764: 760: 4284: 4213: 4107: 3895: 3849: 1516: 1414: 1389: 1165: 134: 3922: 3589: 3528: 3310: 2111:"Ruconest 2100 U powder and solvent for solution for injection" 1402: 1294: 4103: 4031: 4026: 3524: 2749:
Lappin DF, Guc D, Hill A, McShane T, Whaley K (January 1992).
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Overview of all the structural information available in the
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Stoppa-Lyonnet D, Carter PE, Meo T, Tosi M (February 1990).
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negative regulation of complement activation, lectin pathway
3585: 3142:"CpG mutations in the reactive site of human C1 inhibitor" 1204:, may also play a role in the development of age-related 121:, C1IN, C1INH, C1NH, HAE1, HAE2, serpin family G member 1 1378: 1120:
superfamily. Its main function is the inhibition of the
390: 2105: 2103: 4345: 555: 4310: 4247: 4194: 4147: 4086: 3989: 3982: 3956: 3858: 3830: 3623: 3506: 3452: 3401: 3346: 2137:"Ruconest 2100 U powder for solution for injection" 1515: 1488: 1461: 1434: 1429: 1368: 1363: 1328: 1314: 1284: 1246: 1238: 1228: 1223: 1018: 993: 967: 942: 1631: 1629: 1627: 1610: 1608: 1606: 2251:"Summary of product characteristics for Ruconest" 337: 236: 4393:Drugs developed by Takeda Pharmaceutical Company 3575:: Crystal structure of latent human C1-inhibitor 2160: 2158: 1450: 1443: 3097:Aulak KS, Cicardi M, Harrison RA (June 1990). 1786: 1784: 1636:GRCm38: Ensembl release 89: ENSMUSG00000023224 1184:Deficiency of this protein is associated with 3934: 3601: 3540: 3322: 2382:Henry Li H, Riedl M, Kashkin J (April 2019). 1132:C1-inhibitor is the largest member among the 730:negative regulation of endopeptidase activity 8: 2388:Clinical Reviews in Allergy & Immunology 1303: 1216: 750:negative regulation of complement activation 618:serine-type endopeptidase inhibitor activity 1615:GRCh38: Ensembl release 89: ENSG00000149131 1422:In general: ℞ (Prescription only) 1148:domain (also some times referred to as the 3986: 3941: 3927: 3919: 3608: 3594: 3586: 3547: 3533: 3525: 3329: 3315: 3307: 2474:Annals of Allergy, Asthma & Immunology 2082:"Regulatory Decision Summary for Haegarda" 1914:Annals of Allergy, Asthma & Immunology 775: 590: 378: 277: 174: 76: 3909:disorders of globin and globulin proteins 3200: 3157: 3114: 3042: 3032: 2989: 2979: 2936: 2926: 2854: 2774: 2723: 2448: 2399: 2358: 2348: 2307: 2029: 1996:Šimčíková D, Heneberg P (December 2019). 1972: 1715: 1705: 1504: 1497: 685:negative regulation of peptidase activity 720:complement activation, classical pathway 659:collagen-containing extracellular matrix 4352: 3562: 1690:"An overview of the serpin superfamily" 1602: 1477: 1470: 3299:(Plasma protease C1 inhibitor) at the 1731: 1215: 31: 3181:The Journal of Clinical Investigation 2835:The Journal of Clinical Investigation 2225:"Cinryze Monograph for Professionals" 1273: 1266: 1259: 342: 303: 298: 241: 200: 195: 7: 3676:Protein Z-related protease inhibitor 3277: 1793:Springer Seminars in Immunopathology 1351: 1341: 680:blood coagulation, intrinsic pathway 3146:The Journal of Biological Chemistry 1531: 1524: 1234:Cinryze, Ruconest, Berinert, others 740:regulation of complement activation 2886:10.1111/j.1432-1033.1991.tb15911.x 1891:10.1111/j.1432-1033.1991.tb15911.x 1738:: CS1 maint: overridden setting ( 1015: 990: 964: 939: 915: 896: 870: 851: 825: 806: 560: 478: 416: 395: 25: 3671:Plasminogen activator inhibitor-2 3666:Plasminogen activator inhibitor-1 27:Mammalian protein found in humans 4355: 3565: 2874:European Journal of Biochemistry 2337:Patient Preference and Adherence 1879:European Journal of Biochemistry 564: 326: 319: 313: 290: 225: 218: 212: 187: 40: 2170:European Medicines Agency (EMA) 2086:Drug and Health Products Portal 745:regulation of blood coagulation 2596:Behring Institute Mitteilungen 2542:Behring Institute Mitteilungen 2201:. 12 July 2017. Archived from 575:More reference expression data 544:More reference expression data 1: 3159:10.1016/S0021-9258(18)94031-7 2486:10.1016/S1081-1206(10)60585-6 1965:10.1016/S0140-6736(08)61348-3 1926:10.1016/S1081-1206(10)60580-7 1172:) is located on the eleventh 311: 210: 4378:Genes on human chromosome 11 3337:Other hematological agents ( 3232:10.1016/0378-1119(86)90230-1 3116:10.1016/0014-5793(90)81494-9 2725:10.1016/0014-5793(92)80204-T 2198:Food and Drug Administration 1767:10.1358/dnp.2004.17.7.863703 1755:Drug News & Perspectives 649:platelet alpha granule lumen 608:peptidase inhibitor activity 1275:Multum Consumer Information 4409: 4388:Serine protease inhibitors 3886:Thyroxine-binding globulin 3488:Lovotibeglogene autotemcel 3266:gene details page in the 2625:10.1016/j.clim.2004.05.007 2331:Li HH (7 September 2016). 2022:10.1038/s41598-019-54976-4 4210:Decay-accelerating factor 3904: 3560: 2559:Nature Structural Biology 2401:10.1007/s12016-018-8684-1 1805:10.1007/s00281-005-0001-4 1671:"Mouse PubMed Reference:" 1653:"Human PubMed Reference:" 1088: 1083: 1079: 1072: 1056: 1037: 1022: 997: 986: 971: 946: 935: 922: 918: 903: 899: 890: 877: 873: 858: 854: 845: 832: 828: 813: 809: 800: 785: 778: 774: 758: 593: 589: 572: 563: 554: 541: 490: 481: 448:Descending thoracic aorta 428: 419: 389: 381: 377: 360: 347: 310: 289: 280: 276: 259: 246: 209: 186: 177: 173: 128: 125: 115: 108: 103: 84: 79: 62: 57: 52: 48: 39: 34: 3631:Alpha 1-antichymotrypsin 3483:Exagamglogene autotemcel 3473:Betibeglogene autotemcel 2928:10.1073/pnas.87.17.6786 2755:The Biochemical Journal 2510:Pharmacological Reviews 1707:10.1186/gb-2006-7-5-216 1164:The human C1-inhibitor 1043:Chr 11: 57.6 – 57.62 Mb 3832:Cross class inhibitory 2981:10.1073/pnas.87.4.1551 1050:Chr 2: 84.6 – 84.61 Mb 725:innate immune response 705:platelet degranulation 3866:Heat shock protein 47 3034:10.1073/pnas.87.1.265 2450:10.1093/glycob/cwh010 1545:Hereditary angioedema 1186:hereditary angioedema 1110:C1 esterase inhibitor 695:immune system process 644:extracellular exosome 440:right coronary artery 203:Chromosome 11 (human) 18:C1 esterase inhibitor 4249:Complement receptors 3260:genome location and 2480:(1 Suppl 2): S41-6. 2263:on 20 September 2018 1920:(1 Suppl 2): S7-12. 1206:macular degeneration 639:extracellular region 526:white adipose tissue 305:Chromosome 2 (mouse) 167:SERPING1 - orthologs 80:List of PDB id codes 53:Available structures 3661:Protein C inhibitor 3656:Heparin cofactor II 3641:Alpha 2-antiplasmin 3636:Alpha 1-antitrypsin 3455:sickle cell disease 3377:+desoxyribonuclease 3268:UCSC Genome Browser 3025:1990PNAS...87..265L 2972:1990PNAS...87.1551S 2919:1990PNAS...87.6786P 2613:Clinical Immunology 2054:"Genetic disorders" 2014:2019NatSR...918577S 1398:(Prescription only) 1220: 1198:lupus erythematosus 1194:autoimmune diseases 654:extracellular space 634:blood microparticle 432:right lobe of liver 4078:Factor P/Properdin 3983:Activators/enzymes 3368:Desoxyribonuclease 3074:10.1007/BF00195824 2806:10.1007/bf00206079 2683:10.1038/ng0892-354 2571:10.1038/nsb0295-96 2350:10.2147/PPA.S86379 2231:. 23 December 2019 2088:. 1 September 2017 2002:Scientific Reports 1848:10.1007/BF00195824 1114:protease inhibitor 880:ENSMUSG00000023224 668:Biological process 627:Cellular component 601:Molecular function 494:left lobe of liver 4383:Complement system 4343: 4342: 4190: 4189: 3950:Complement system 3916: 3915: 3583: 3582: 3522: 3521: 3193:10.1172/JCI114095 2847:10.1172/JCI115373 2767:10.1042/bj2810437 2654:10.1159/000154075 2172:. 28 October 2010 1959:(9652): 1828–34. 1542: 1541: 1418: 1406: 1393: 1381: 1297: 1242:RVG-19303, CSL830 1122:complement system 1116:belonging to the 1099: 1098: 1095: 1094: 1068: 1067: 1033: 1032: 1012: 1011: 982: 981: 961: 960: 931: 930: 912: 911: 886: 885: 867: 866: 841: 840: 822: 821: 770: 769: 735:blood coagulation 710:blood circulation 585: 584: 581: 580: 550: 549: 537: 536: 475: 474: 464:left uterine tube 373: 372: 272: 271: 99: 98: 95: 94: 63:Ortholog search: 16:(Redirected from 4400: 4360: 4359: 4358: 4351: 4324:immune adherence 3987: 3943: 3936: 3929: 3920: 3610: 3603: 3596: 3587: 3569: 3549: 3542: 3535: 3526: 3460:beta thalassemia 3331: 3324: 3317: 3308: 3281: 3243: 3214: 3204: 3171: 3161: 3136: 3118: 3093: 3056: 3046: 3036: 3003: 2993: 2983: 2950: 2940: 2930: 2897: 2868: 2858: 2825: 2788: 2778: 2761:(Pt 2): 437–42. 2745: 2727: 2702: 2665: 2636: 2607: 2590: 2553: 2526: 2525: 2504: 2498: 2497: 2469: 2463: 2462: 2452: 2428: 2422: 2421: 2403: 2379: 2373: 2372: 2362: 2352: 2328: 2322: 2321: 2311: 2300:10.1002/nop2.194 2279: 2273: 2272: 2270: 2268: 2262: 2256:. Archived from 2255: 2247: 2241: 2240: 2238: 2236: 2221: 2215: 2214: 2212: 2210: 2188: 2182: 2181: 2179: 2177: 2162: 2153: 2152: 2150: 2148: 2143:. 30 August 2023 2133: 2127: 2126: 2124: 2122: 2117:. 30 August 2023 2107: 2098: 2097: 2095: 2093: 2078: 2072: 2071: 2069: 2067: 2050: 2044: 2043: 2033: 1993: 1987: 1986: 1976: 1944: 1938: 1937: 1909: 1903: 1902: 1874: 1868: 1867: 1831: 1825: 1824: 1788: 1779: 1778: 1750: 1744: 1743: 1737: 1729: 1719: 1709: 1685: 1679: 1678: 1667: 1661: 1660: 1649: 1643: 1633: 1622: 1612: 1578:Escherichia coli 1575:technology, but 1535: 1528: 1508: 1501: 1481: 1474: 1454: 1447: 1416: 1413: 1404: 1401: 1391: 1388: 1380: 1377: 1355: 1345: 1307: 1296: 1293: 1277: 1270: 1263: 1221: 1219: 1196:, most markedly 1081: 1080: 1052: 1045: 1028: 1016: 1007: 991: 987:RefSeq (protein) 977: 965: 956: 940: 916: 897: 871: 852: 826: 807: 776: 591: 577: 568: 561: 546: 522:efferent ductule 486: 484:Top expressed in 479: 456:popliteal artery 424: 422:Top expressed in 417: 396: 379: 369: 356: 345: 330: 323: 317: 306: 294: 278: 268: 255: 244: 229: 222: 216: 205: 191: 175: 169: 120: 113: 90: 77: 71: 50: 49: 44: 32: 21: 4408: 4407: 4403: 4402: 4401: 4399: 4398: 4397: 4368: 4367: 4366: 4356: 4354: 4346: 4344: 4339: 4306: 4243: 4186: 4143: 4082: 3978: 3952: 3947: 3917: 3912: 3900: 3891:Angiotensinogen 3854: 3826: 3619: 3614: 3584: 3579: 3576: 3570: 3556: 3553: 3523: 3518: 3502: 3457: 3448: 3405: 3397: 3342: 3335: 3251: 3246: 3217: 3174: 3139: 3096: 3059: 3006: 2953: 2913:(17): 6786–90. 2900: 2871: 2828: 2791: 2748: 2705: 2671:Nature Genetics 2668: 2639: 2610: 2593: 2556: 2539: 2535: 2533:Further reading 2530: 2529: 2506: 2505: 2501: 2471: 2470: 2466: 2430: 2429: 2425: 2381: 2380: 2376: 2330: 2329: 2325: 2281: 2280: 2276: 2266: 2264: 2260: 2253: 2249: 2248: 2244: 2234: 2232: 2223: 2222: 2218: 2208: 2206: 2205:on 22 July 2017 2190: 2189: 2185: 2175: 2173: 2166:"Ruconest EPAR" 2164: 2163: 2156: 2146: 2144: 2135: 2134: 2130: 2120: 2118: 2109: 2108: 2101: 2091: 2089: 2080: 2079: 2075: 2065: 2063: 2052: 2051: 2047: 1995: 1994: 1990: 1946: 1945: 1941: 1911: 1910: 1906: 1876: 1875: 1871: 1833: 1832: 1828: 1790: 1789: 1782: 1752: 1751: 1747: 1730: 1687: 1686: 1682: 1669: 1668: 1664: 1651: 1650: 1646: 1634: 1625: 1613: 1604: 1599: 1591: 1565: 1547: 1538: 1511: 1484: 1457: 1425: 1359: 1317: 1310: 1280: 1214: 1182: 1180:Role in disease 1176:(11q11-q13.1). 1162: 1150:N-terminal tail 1140:structure. The 1130: 1090:View/Edit Mouse 1085:View/Edit Human 1048: 1041: 1038:Location (UCSC) 1024: 1003: 999: 973: 952: 948: 861:ENSG00000149131 754: 663: 622: 613:protein binding 573: 542: 533: 528: 524: 520: 518:right lung lobe 516: 512: 508: 504: 500: 496: 482: 471: 466: 462: 460:tibial arteries 458: 454: 452:ascending aorta 450: 446: 442: 438: 434: 420: 364: 351: 343: 333: 332: 331: 324: 304: 281:Gene location ( 263: 250: 242: 232: 231: 230: 223: 201: 178:Gene location ( 129: 116: 109: 86: 64: 28: 23: 22: 15: 12: 11: 5: 4406: 4404: 4396: 4395: 4390: 4385: 4380: 4370: 4369: 4365: 4364: 4341: 4340: 4338: 4337: 4332: 4326: 4321: 4314: 4312: 4308: 4307: 4305: 4304: 4303: 4302: 4297: 4287: 4274: 4269: 4264: 4259: 4253: 4251: 4245: 4244: 4242: 4241: 4232: 4231: 4222: 4221: 4216: 4207: 4198: 4196: 4192: 4191: 4188: 4187: 4185: 4184: 4183: 4182: 4177: 4172: 4167: 4162: 4151: 4149: 4145: 4144: 4142: 4141: 4136: 4130: 4129: 4128: 4127: 4122: 4112: 4111: 4110: 4101: 4090: 4088: 4084: 4083: 4081: 4080: 4075: 4070: 4061: 4060: 4055: 4042: 4041: 4036: 4035: 4034: 4029: 4019: 4018: 4017: 4012: 4007: 3993: 3991: 3984: 3980: 3979: 3977: 3976: 3971: 3966: 3960: 3958: 3954: 3953: 3948: 3946: 3945: 3938: 3931: 3923: 3914: 3913: 3905: 3902: 3901: 3899: 3898: 3893: 3888: 3883: 3878: 3873: 3868: 3862: 3860: 3856: 3855: 3853: 3852: 3847: 3842: 3836: 3834: 3828: 3827: 3825: 3824: 3819: 3814: 3809: 3804: 3799: 3794: 3789: 3784: 3779: 3773: 3772: 3767: 3762: 3757: 3752: 3747: 3742: 3737: 3732: 3727: 3722: 3716: 3715: 3710: 3705: 3700: 3695: 3690: 3685: 3679: 3678: 3673: 3668: 3663: 3658: 3653: 3648: 3643: 3638: 3633: 3627: 3625: 3621: 3620: 3615: 3613: 3612: 3605: 3598: 3590: 3581: 3580: 3578: 3577: 3571: 3564: 3561: 3558: 3557: 3554: 3552: 3551: 3544: 3537: 3529: 3520: 3519: 3517: 3516: 3510: 3508: 3504: 3503: 3501: 3500: 3495: 3490: 3485: 3480: 3475: 3469: 3467: 3453:Drugs used in 3450: 3449: 3447: 3446: 3441: 3436: 3431: 3426: 3421: 3415: 3413: 3402:Drugs used in 3399: 3398: 3396: 3395: 3390: 3385: 3380: 3370: 3365: 3360: 3354: 3352: 3344: 3343: 3336: 3334: 3333: 3326: 3319: 3311: 3305: 3304: 3283: 3271: 3250: 3249:External links 3247: 3245: 3244: 3215: 3187:(6): 1888–93. 3172: 3152:(6): 3066–71. 3137: 3094: 3062:Human Genetics 3057: 3004: 2951: 2898: 2869: 2826: 2794:Human Genetics 2789: 2746: 2703: 2666: 2642:Human Heredity 2637: 2608: 2598:(93): 313–20. 2591: 2554: 2544:(84): 180–92. 2536: 2534: 2531: 2528: 2527: 2499: 2464: 2423: 2394:(2): 207–218. 2374: 2323: 2294:(1): 126–135. 2274: 2242: 2216: 2183: 2154: 2128: 2099: 2073: 2045: 1988: 1939: 1904: 1869: 1836:Human Genetics 1826: 1780: 1745: 1694:Genome Biology 1680: 1662: 1644: 1623: 1601: 1600: 1598: 1595: 1590: 1587: 1564: 1561: 1546: 1543: 1540: 1539: 1537: 1536: 1529: 1521: 1519: 1513: 1512: 1510: 1509: 1502: 1494: 1492: 1486: 1485: 1483: 1482: 1475: 1467: 1465: 1459: 1458: 1456: 1455: 1448: 1440: 1438: 1432: 1431: 1427: 1426: 1424: 1423: 1420: 1411: 1399: 1386: 1374: 1372: 1366: 1365: 1361: 1360: 1358: 1357: 1334: 1332: 1326: 1325: 1320: 1318:administration 1312: 1311: 1309: 1308: 1290: 1288: 1282: 1281: 1279: 1278: 1271: 1264: 1256: 1254: 1244: 1243: 1240: 1236: 1235: 1232: 1226: 1225: 1213: 1210: 1181: 1178: 1161: 1158: 1129: 1126: 1097: 1096: 1093: 1092: 1087: 1077: 1076: 1070: 1069: 1066: 1065: 1063: 1061: 1054: 1053: 1046: 1039: 1035: 1034: 1031: 1030: 1020: 1019: 1013: 1010: 1009: 995: 994: 988: 984: 983: 980: 979: 969: 968: 962: 959: 958: 944: 943: 937: 933: 932: 929: 928: 920: 919: 913: 910: 909: 901: 900: 894: 888: 887: 884: 883: 875: 874: 868: 865: 864: 856: 855: 849: 843: 842: 839: 838: 830: 829: 823: 820: 819: 811: 810: 804: 798: 797: 792: 787: 783: 782: 772: 771: 768: 767: 756: 755: 753: 752: 747: 742: 737: 732: 727: 722: 717: 712: 707: 702: 697: 692: 687: 682: 677: 671: 669: 665: 664: 662: 661: 656: 651: 646: 641: 636: 630: 628: 624: 623: 621: 620: 615: 610: 604: 602: 598: 597: 587: 586: 583: 582: 579: 578: 570: 569: 558: 552: 551: 548: 547: 539: 538: 535: 534: 532: 531: 527: 523: 519: 515: 511: 507: 503: 499: 495: 491: 488: 487: 476: 473: 472: 470: 469: 465: 461: 457: 453: 449: 445: 444:gastric mucosa 441: 437: 433: 429: 426: 425: 413: 412: 404: 393: 387: 386: 383:RNA expression 375: 374: 371: 370: 362: 358: 357: 349: 346: 341: 335: 334: 325: 318: 312: 308: 307: 302: 296: 295: 287: 286: 274: 273: 270: 269: 261: 257: 256: 248: 245: 240: 234: 233: 224: 217: 211: 207: 206: 199: 193: 192: 184: 183: 171: 170: 127: 123: 122: 114: 106: 105: 101: 100: 97: 96: 93: 92: 82: 81: 73: 72: 61: 55: 54: 46: 45: 37: 36: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 4405: 4394: 4391: 4389: 4386: 4384: 4381: 4379: 4376: 4375: 4373: 4363: 4353: 4349: 4336: 4333: 4331: 4327: 4325: 4322: 4319: 4316: 4315: 4313: 4309: 4301: 4298: 4296: 4293: 4292: 4291: 4290:Anaphylatoxin 4288: 4286: 4282: 4278: 4275: 4273: 4270: 4268: 4265: 4263: 4260: 4258: 4255: 4254: 4252: 4250: 4246: 4240: 4237: 4234: 4233: 4230: 4227: 4224: 4223: 4220: 4217: 4215: 4211: 4208: 4206: 4203: 4200: 4199: 4197: 4193: 4181: 4178: 4176: 4173: 4171: 4168: 4166: 4163: 4161: 4158: 4157: 4156: 4153: 4152: 4150: 4146: 4140: 4139:C5-convertase 4137: 4135: 4134:C3-convertase 4132: 4131: 4126: 4123: 4121: 4118: 4117: 4116: 4113: 4109: 4105: 4102: 4100: 4097: 4096: 4095: 4092: 4091: 4089: 4085: 4079: 4076: 4074: 4071: 4069: 4066: 4063: 4062: 4059: 4056: 4054: 4050: 4047: 4044: 4043: 4040: 4037: 4033: 4030: 4028: 4025: 4024: 4023: 4020: 4016: 4013: 4011: 4008: 4006: 4003: 4002: 4001: 3998: 3995: 3994: 3992: 3988: 3985: 3981: 3975: 3972: 3970: 3967: 3965: 3962: 3961: 3959: 3955: 3951: 3944: 3939: 3937: 3932: 3930: 3925: 3924: 3921: 3911: 3910: 3903: 3897: 3894: 3892: 3889: 3887: 3884: 3882: 3879: 3877: 3874: 3872: 3869: 3867: 3864: 3863: 3861: 3859:noninhibitory 3857: 3851: 3848: 3846: 3843: 3841: 3838: 3837: 3835: 3833: 3829: 3823: 3820: 3818: 3815: 3813: 3810: 3808: 3805: 3803: 3800: 3798: 3795: 3793: 3790: 3788: 3785: 3783: 3780: 3778: 3775: 3774: 3771: 3768: 3766: 3763: 3761: 3758: 3756: 3753: 3751: 3748: 3746: 3743: 3741: 3738: 3736: 3733: 3731: 3728: 3726: 3723: 3721: 3718: 3717: 3714: 3711: 3709: 3706: 3704: 3701: 3699: 3696: 3694: 3691: 3689: 3686: 3684: 3681: 3680: 3677: 3674: 3672: 3669: 3667: 3664: 3662: 3659: 3657: 3654: 3652: 3649: 3647: 3644: 3642: 3639: 3637: 3634: 3632: 3629: 3628: 3626: 3622: 3618: 3611: 3606: 3604: 3599: 3597: 3592: 3591: 3588: 3574: 3568: 3563: 3559: 3550: 3545: 3543: 3538: 3536: 3531: 3530: 3527: 3515: 3512: 3511: 3509: 3505: 3499: 3496: 3494: 3491: 3489: 3486: 3484: 3481: 3479: 3478:Crizanlizumab 3476: 3474: 3471: 3470: 3468: 3465: 3461: 3456: 3451: 3445: 3442: 3440: 3437: 3435: 3432: 3430: 3429:Conestat alfa 3427: 3425: 3422: 3420: 3417: 3416: 3414: 3411: 3407: 3400: 3394: 3391: 3389: 3388:Streptokinase 3386: 3384: 3383:Hyaluronidase 3381: 3378: 3374: 3371: 3369: 3366: 3364: 3361: 3359: 3356: 3355: 3353: 3350: 3345: 3340: 3332: 3327: 3325: 3320: 3318: 3313: 3312: 3309: 3302: 3298: 3297: 3292: 3288: 3284: 3280: 3275: 3272: 3269: 3265: 3264: 3259: 3258: 3253: 3252: 3248: 3241: 3237: 3233: 3229: 3226:(3): 265–72. 3225: 3221: 3216: 3212: 3208: 3203: 3198: 3194: 3190: 3186: 3182: 3178: 3173: 3169: 3165: 3160: 3155: 3151: 3147: 3143: 3138: 3134: 3130: 3126: 3122: 3117: 3112: 3109:(1–2): 13–6. 3108: 3104: 3100: 3095: 3091: 3087: 3083: 3079: 3075: 3071: 3067: 3063: 3058: 3054: 3050: 3045: 3040: 3035: 3030: 3026: 3022: 3018: 3014: 3010: 3005: 3001: 2997: 2992: 2987: 2982: 2977: 2973: 2969: 2966:(4): 1551–5. 2965: 2961: 2957: 2952: 2948: 2944: 2939: 2934: 2929: 2924: 2920: 2916: 2912: 2908: 2904: 2899: 2895: 2891: 2887: 2883: 2879: 2875: 2870: 2866: 2862: 2857: 2852: 2848: 2844: 2840: 2836: 2832: 2827: 2823: 2819: 2815: 2811: 2807: 2803: 2799: 2795: 2790: 2786: 2782: 2777: 2772: 2768: 2764: 2760: 2756: 2752: 2747: 2743: 2739: 2735: 2731: 2726: 2721: 2717: 2713: 2709: 2704: 2700: 2696: 2692: 2688: 2684: 2680: 2676: 2672: 2667: 2663: 2659: 2655: 2651: 2647: 2643: 2638: 2634: 2630: 2626: 2622: 2618: 2614: 2609: 2605: 2601: 2597: 2592: 2588: 2584: 2580: 2576: 2572: 2568: 2565:(2): 96–113. 2564: 2560: 2555: 2551: 2547: 2543: 2538: 2537: 2532: 2523: 2519: 2516:(1): 91–112. 2515: 2511: 2503: 2500: 2495: 2491: 2487: 2483: 2479: 2475: 2468: 2465: 2460: 2456: 2451: 2446: 2442: 2438: 2434: 2427: 2424: 2419: 2415: 2411: 2407: 2402: 2397: 2393: 2389: 2385: 2378: 2375: 2370: 2366: 2361: 2356: 2351: 2346: 2342: 2338: 2334: 2327: 2324: 2319: 2315: 2310: 2305: 2301: 2297: 2293: 2289: 2285: 2278: 2275: 2259: 2252: 2246: 2243: 2230: 2226: 2220: 2217: 2204: 2200: 2199: 2193: 2187: 2184: 2171: 2167: 2161: 2159: 2155: 2142: 2138: 2132: 2129: 2116: 2112: 2106: 2104: 2100: 2087: 2083: 2077: 2074: 2061: 2060: 2059:Health Canada 2055: 2049: 2046: 2041: 2037: 2032: 2027: 2023: 2019: 2015: 2011: 2007: 2003: 1999: 1992: 1989: 1984: 1980: 1975: 1970: 1966: 1962: 1958: 1954: 1950: 1943: 1940: 1935: 1931: 1927: 1923: 1919: 1915: 1908: 1905: 1900: 1896: 1892: 1888: 1884: 1880: 1873: 1870: 1865: 1861: 1857: 1853: 1849: 1845: 1841: 1837: 1830: 1827: 1822: 1818: 1814: 1810: 1806: 1802: 1799:(3): 286–98. 1798: 1794: 1787: 1785: 1781: 1776: 1772: 1768: 1764: 1761:(7): 439–46. 1760: 1756: 1749: 1746: 1741: 1735: 1727: 1723: 1718: 1713: 1708: 1703: 1699: 1695: 1691: 1684: 1681: 1676: 1672: 1666: 1663: 1658: 1654: 1648: 1645: 1641: 1637: 1632: 1630: 1628: 1624: 1620: 1616: 1611: 1609: 1607: 1603: 1596: 1594: 1588: 1586: 1584: 1580: 1579: 1574: 1570: 1569:donated blood 1562: 1560: 1557: 1554: 1552: 1544: 1534: 1530: 1527: 1523: 1522: 1520: 1518: 1514: 1507: 1503: 1500: 1496: 1495: 1493: 1491: 1487: 1480: 1476: 1473: 1469: 1468: 1466: 1464: 1460: 1453: 1449: 1446: 1442: 1441: 1439: 1437: 1433: 1428: 1421: 1419: Rx-only 1412: 1410: 1400: 1397: 1387: 1385: 1376: 1375: 1373: 1371: 1367: 1362: 1354: 1349: 1344: 1339: 1336: 1335: 1333: 1331: 1327: 1324: 1321: 1319: 1313: 1306: 1301: 1292: 1291: 1289: 1287: 1283: 1276: 1272: 1269: 1265: 1262: 1258: 1257: 1255: 1253: 1249: 1245: 1241: 1237: 1233: 1231: 1227: 1224:Clinical data 1222: 1211: 1209: 1207: 1203: 1199: 1195: 1191: 1187: 1179: 1177: 1175: 1171: 1167: 1159: 1157: 1155: 1151: 1147: 1143: 1139: 1135: 1127: 1125: 1123: 1119: 1115: 1111: 1107: 1103: 1091: 1086: 1082: 1078: 1075: 1071: 1064: 1062: 1059: 1055: 1051: 1047: 1044: 1040: 1036: 1029: 1027: 1021: 1017: 1014: 1008: 1006: 1002: 996: 992: 989: 985: 978: 976: 970: 966: 963: 957: 955: 951: 945: 941: 938: 936:RefSeq (mRNA) 934: 927: 926: 921: 917: 914: 908: 907: 902: 898: 895: 893: 889: 882: 881: 876: 872: 869: 863: 862: 857: 853: 850: 848: 844: 837: 836: 831: 827: 824: 818: 817: 812: 808: 805: 803: 799: 796: 793: 791: 788: 784: 781: 777: 773: 766: 762: 757: 751: 748: 746: 743: 741: 738: 736: 733: 731: 728: 726: 723: 721: 718: 716: 713: 711: 708: 706: 703: 701: 698: 696: 693: 691: 688: 686: 683: 681: 678: 676: 673: 672: 670: 667: 666: 660: 657: 655: 652: 650: 647: 645: 642: 640: 637: 635: 632: 631: 629: 626: 625: 619: 616: 614: 611: 609: 606: 605: 603: 600: 599: 596: 595:Gene ontology 592: 588: 576: 571: 567: 562: 559: 557: 553: 545: 540: 529: 525: 521: 517: 513: 509: 505: 501: 497: 493: 492: 489: 485: 480: 477: 467: 463: 459: 455: 451: 447: 443: 439: 435: 431: 430: 427: 423: 418: 415: 414: 411: 409: 405: 403: 402: 398: 397: 394: 392: 388: 384: 380: 376: 368: 363: 359: 355: 350: 340: 336: 329: 322: 316: 309: 301: 297: 293: 288: 284: 279: 275: 267: 262: 258: 254: 249: 239: 235: 228: 221: 215: 208: 204: 198: 194: 190: 185: 181: 176: 172: 168: 164: 160: 156: 152: 148: 144: 140: 136: 132: 124: 119: 112: 107: 102: 91: 89: 83: 78: 75: 74: 70: 67: 60: 56: 51: 47: 43: 38: 33: 30: 19: 4335:Opsonization 4330:inflammation 4318:Cytotoxicity 4235: 4225: 4205:C1-inhibitor 4204: 4201: 4064: 4045: 3996: 3906: 3806: 3651:C1-inhibitor 3650: 3646:Antithrombin 3572: 3424:C1-inhibitor 3423: 3419:Berotralstat 3373:Fibrinolysin 3363:Chymotrypsin 3294: 3262: 3256: 3223: 3219: 3184: 3180: 3149: 3145: 3106: 3103:FEBS Letters 3102: 3068:(5): 477–9. 3065: 3061: 3019:(1): 265–8. 3016: 3012: 2963: 2959: 2910: 2906: 2880:(2): 301–8. 2877: 2873: 2841:(3): 755–9. 2838: 2834: 2800:(2): 231–2. 2797: 2793: 2758: 2754: 2715: 2712:FEBS Letters 2711: 2677:(5): 354–8. 2674: 2670: 2648:(4): 231–4. 2645: 2641: 2616: 2612: 2595: 2562: 2558: 2541: 2513: 2509: 2502: 2477: 2473: 2467: 2443:(1): 51–64. 2440: 2437:Glycobiology 2436: 2426: 2391: 2387: 2377: 2340: 2336: 2326: 2291: 2288:Nursing Open 2287: 2277: 2265:. Retrieved 2258:the original 2245: 2233:. Retrieved 2228: 2219: 2207:. Retrieved 2203:the original 2195: 2186: 2174:. Retrieved 2169: 2145:. Retrieved 2140: 2131: 2119:. Retrieved 2114: 2090:. Retrieved 2085: 2076: 2064:. Retrieved 2062:. 9 May 2018 2057: 2048: 2008:(1): 18577. 2005: 2001: 1991: 1956: 1952: 1942: 1917: 1913: 1907: 1885:(2): 301–8. 1882: 1878: 1872: 1842:(5): 477–9. 1839: 1835: 1829: 1796: 1792: 1758: 1754: 1748: 1734:cite journal 1697: 1693: 1683: 1674: 1665: 1656: 1647: 1592: 1576: 1566: 1558: 1555: 1550: 1548: 1445:1018837-94-9 1370:Legal status 1364:Legal status 1286:License data 1201: 1183: 1169: 1163: 1154:glycosylated 1149: 1133: 1131: 1109: 1105: 1102:C1-inhibitor 1101: 1100: 1023: 1005:NP_001027466 998: 972: 950:NM_001032295 947: 923: 904: 878: 859: 833: 814: 794: 789: 700:human ageing 690:fibrinolysis 502:carotid body 406: 399: 126:External IDs 85: 29: 3881:Transcortin 3555:PDB gallery 3444:Lanadelumab 3434:Ecallantide 2718:(1): 34–6. 2343:: 1727–37. 1583:orphan drug 1573:recombinant 1430:Identifiers 1323:Intravenous 1305:C1 esterase 1239:Other names 1230:Trade names 1218:C1 esterase 1212:Medical use 468:right ovary 365:84,605,788 352:84,595,731 264:57,619,171 251:57,597,387 104:Identifiers 4372:Categories 4195:Inhibitors 3624:inhibitory 3406:angioedema 3404:hereditary 2619:(1): 3–9. 2267:9 February 1700:(5): 216. 1642:, May 2017 1621:, May 2017 1597:References 1506:5QS67N4551 1499:6KIC4BB60G 1452:80295-38-1 1436:CAS Number 1190:bradykinin 1174:chromosome 1146:N-terminal 1142:C-terminal 1128:Proteomics 675:hemostasis 514:right lung 436:right lung 410:(ortholog) 344:2|2 D 147:HomoloGene 4328:Inducing 3907:see also 3876:Ovalbumin 3770:SERPINB13 3765:SERPINB10 3713:SERPINA14 3498:Voxelotor 3493:Mitapivat 3439:Icatibant 3358:Bromelain 3347:Enzymes ( 2229:Drugs.com 2192:"Cinryze" 2176:30 August 2147:30 August 2121:30 August 1563:Synthesis 1316:Routes of 1268:Monograph 1261:Monograph 1252:Drugs.com 1026:NP_033906 1001:NP_000053 975:NM_009776 954:NM_000062 780:Orthologs 155:GeneCards 4362:Medicine 4320:(by MAC) 4311:Function 4239:Factor H 4219:Factor I 4073:Factor D 4068:Factor B 3957:Pathways 3822:SERPINI2 3817:SERPINI1 3812:SERPINH1 3807:SERPING1 3802:SERPINF1 3797:SERPINE2 3792:SERPINE2 3787:SERPINE1 3782:SERPIND1 3777:SERPINC1 3760:SERPINB9 3755:SERPINB8 3750:SERPINB7 3745:SERPINB6 3740:SERPINB5 3735:SERPINB4 3730:SERPINB3 3725:SERPINB2 3720:SERPINB1 3708:SERPINA9 3703:SERPINA5 3698:SERPINA4 3693:SERPINA3 3688:SERPINA2 3683:SERPINA1 3263:SERPING1 3257:SERPING1 3133:35981265 3090:21989261 2822:20492891 2742:28082291 2699:29076504 2633:15596403 2587:21223825 2522:10699156 2494:18220151 2459:14514717 2418:49269933 2410:29909591 2369:27660422 2318:30534402 2235:20 April 2209:20 April 2092:13 April 2066:13 April 2040:31819097 1983:18842294 1934:18220146 1864:21989261 1821:24583403 1813:16267649 1775:15514703 1726:16737556 1638:– 1617:– 1589:Research 1463:DrugBank 1330:ATC code 1300:DailyMed 1202:SERPING1 1170:SERPING1 1160:Genetics 1074:Wikidata 759:Sources: 498:gastrula 159:SERPING1 118:SERPING1 35:SERPING1 3617:Serpins 3393:Trypsin 3301:PDBe-KB 3291:UniProt 3276: ( 3274:B02AB03 3240:3089875 3211:2723063 3168:2563376 3125:2365061 3082:2323781 3053:2296585 3021:Bibcode 3000:2154751 2968:Bibcode 2947:2118657 2915:Bibcode 2894:2026152 2865:1885769 2814:1684567 2785:1531292 2776:1130704 2734:1451784 2691:1363816 2662:1339401 2604:8172583 2579:7749926 2550:2478116 2360:5019432 2309:6279717 2031:6901466 2010:Bibcode 1974:5983350 1899:2026152 1856:2323781 1717:1779521 1640:Ensembl 1619:Ensembl 1551:Cinryze 1479:DB09228 1472:DB06404 1382:: 1350: ( 1348:B06AC04 1346:) 1340: ( 1338:B06AC01 1302::  1112:) is a 892:UniProt 847:Ensembl 786:Species 765:QuickGO 506:decidua 385:pattern 243:11q12.1 111:Aliases 4348:Portal 4087:Middle 3871:Maspin 3845:SCCA-1 3507:Others 3296:P05155 3254:Human 3238:  3209:  3202:303909 3199:  3166:  3131:  3123:  3088:  3080:  3051:  3041:  2998:  2988:  2945:  2935:  2892:  2863:  2856:295456 2853:  2820:  2812:  2783:  2773:  2740:  2732:  2697:  2689:  2660:  2631:  2602:  2585:  2577:  2548:  2520:  2492:  2457:  2416:  2408:  2367:  2357:  2316:  2306:  2038:  2028:  1981:  1971:  1953:Lancet 1932:  1897:  1862:  1854:  1819:  1811:  1773:  1724:  1714:  1533:D10845 1526:D08780 1409:℞-only 1407: 1394: 1384:℞-only 1298:  1138:domain 1134:serpin 1118:serpin 1106:C1-inh 1060:search 1058:PubMed 925:P97290 906:P05155 802:Entrez 556:BioGPS 530:uterus 143:894696 135:606860 4281:CD11c 4277:CD11b 4053:MASP2 4049:MASP1 3990:Early 3514:Hemin 3464:B06AX 3410:B06AC 3349:B06AA 3129:S2CID 3086:S2CID 3044:53243 2991:53513 2938:54622 2818:S2CID 2738:S2CID 2695:S2CID 2583:S2CID 2414:S2CID 2261:(PDF) 2254:(PDF) 2196:U.S. 2141:(emc) 2115:(emc) 1860:S2CID 1817:S2CID 835:12258 795:Mouse 790:Human 761:Amigo 510:ankle 408:Mouse 401:Human 348:Start 283:Mouse 247:Start 180:Human 4285:CD18 4229:C4BP 4214:CD59 4202:CLA: 4148:Late 4108:iC3b 3896:PEDF 3850:CrmA 3840:MENT 3573:2oay 3458:and 3289:for 3236:PMID 3220:Gene 3207:PMID 3164:PMID 3121:PMID 3078:PMID 3049:PMID 2996:PMID 2943:PMID 2890:PMID 2861:PMID 2810:PMID 2781:PMID 2730:PMID 2687:PMID 2658:PMID 2629:PMID 2600:PMID 2575:PMID 2546:PMID 2518:PMID 2490:PMID 2455:PMID 2406:PMID 2365:PMID 2314:PMID 2269:2012 2237:2020 2211:2020 2178:2024 2149:2024 2123:2024 2094:2024 2068:2024 2036:PMID 1979:PMID 1930:PMID 1895:PMID 1852:PMID 1809:PMID 1771:PMID 1740:link 1722:PMID 1517:KEGG 1490:UNII 1248:AHFS 1166:gene 391:Bgee 339:Band 300:Chr. 238:Band 197:Chr. 131:OMIM 88:2OAY 69:RCSB 66:PDBe 4300:C5a 4295:C3a 4272:CR4 4267:CR3 4262:CR2 4257:CR1 4226:CL: 4160:C5b 4155:MAC 4125:C5b 4120:C5a 4104:C3b 4099:C3a 4058:MBL 4032:C4b 4027:C4a 4015:C1s 4010:C1r 4005:C1q 3339:B06 3287:PDB 3279:WHO 3228:doi 3197:PMC 3189:doi 3154:doi 3150:264 3111:doi 3107:266 3070:doi 3039:PMC 3029:doi 2986:PMC 2976:doi 2933:PMC 2923:doi 2882:doi 2878:197 2851:PMC 2843:doi 2802:doi 2771:PMC 2763:doi 2759:281 2720:doi 2716:301 2679:doi 2650:doi 2621:doi 2617:114 2567:doi 2482:doi 2478:100 2445:doi 2396:doi 2355:PMC 2345:doi 2304:PMC 2296:doi 2026:PMC 2018:doi 1969:PMC 1961:doi 1957:372 1922:doi 1918:100 1887:doi 1883:197 1844:doi 1801:doi 1763:doi 1712:PMC 1702:doi 1396:POM 1353:WHO 1343:WHO 816:710 361:End 260:End 163:OMA 139:MGI 59:PDB 4374:: 4236:A: 4180:C9 4175:C8 4170:C7 4165:C6 4115:C5 4094:C3 4065:A: 4046:L: 4039:C2 4022:C4 4000:C1 3997:C: 3293:: 3234:. 3224:42 3222:. 3205:. 3195:. 3185:83 3183:. 3179:. 3162:. 3148:. 3144:. 3127:. 3119:. 3105:. 3101:. 3084:. 3076:. 3066:84 3064:. 3047:. 3037:. 3027:. 3017:87 3015:. 3011:. 2994:. 2984:. 2974:. 2964:87 2962:. 2958:. 2941:. 2931:. 2921:. 2911:87 2909:. 2905:. 2888:. 2876:. 2859:. 2849:. 2839:88 2837:. 2833:. 2816:. 2808:. 2798:88 2796:. 2779:. 2769:. 2757:. 2753:. 2736:. 2728:. 2714:. 2710:. 2693:. 2685:. 2673:. 2656:. 2646:42 2644:. 2627:. 2615:. 2581:. 2573:. 2561:. 2514:52 2512:. 2488:. 2476:. 2453:. 2441:14 2439:. 2435:. 2412:. 2404:. 2392:56 2390:. 2386:. 2363:. 2353:. 2341:10 2339:. 2335:. 2312:. 2302:. 2290:. 2286:. 2227:. 2194:. 2168:. 2157:^ 2139:. 2113:. 2102:^ 2084:. 2056:. 2034:. 2024:. 2016:. 2004:. 2000:. 1977:. 1967:. 1955:. 1951:. 1928:. 1916:. 1893:. 1881:. 1858:. 1850:. 1840:84 1838:. 1815:. 1807:. 1797:27 1795:. 1783:^ 1769:. 1759:17 1757:. 1736:}} 1732:{{ 1720:. 1710:. 1696:. 1692:. 1673:. 1655:. 1626:^ 1605:^ 1415:EU 1403:US 1390:UK 1379:CA 1295:US 1108:, 763:/ 367:bp 354:bp 266:bp 253:bp 161:; 157:: 153:; 151:44 149:: 145:; 141:: 137:; 133:: 4350:: 4283:/ 4279:/ 4212:/ 4106:/ 4051:/ 3974:A 3969:L 3964:C 3942:e 3935:t 3928:v 3609:e 3602:t 3595:v 3548:e 3541:t 3534:v 3466:) 3462:( 3412:) 3408:( 3379:) 3375:( 3351:) 3341:) 3330:e 3323:t 3316:v 3303:. 3282:) 3270:. 3242:. 3230:: 3213:. 3191:: 3170:. 3156:: 3135:. 3113:: 3092:. 3072:: 3055:. 3031:: 3023:: 3002:. 2978:: 2970:: 2949:. 2925:: 2917:: 2896:. 2884:: 2867:. 2845:: 2824:. 2804:: 2787:. 2765:: 2744:. 2722:: 2701:. 2681:: 2675:1 2664:. 2652:: 2635:. 2623:: 2606:. 2589:. 2569:: 2563:2 2552:. 2524:. 2496:. 2484:: 2461:. 2447:: 2420:. 2398:: 2371:. 2347:: 2320:. 2298:: 2292:6 2271:. 2239:. 2213:. 2180:. 2151:. 2125:. 2096:. 2070:. 2042:. 2020:: 2012:: 2006:9 1985:. 1963:: 1936:. 1924:: 1901:. 1889:: 1866:. 1846:: 1823:. 1803:: 1777:. 1765:: 1742:) 1728:. 1704:: 1698:7 1677:. 1659:. 1417:: 1405:: 1392:: 1356:) 1250:/ 1168:( 1104:( 285:) 182:) 165:: 20:)

Index

C1 esterase inhibitor

PDB
PDBe
RCSB
2OAY
Aliases
SERPING1
OMIM
606860
MGI
894696
HomoloGene
44
GeneCards
SERPING1
OMA
SERPING1 - orthologs
Human
Chromosome 11 (human)
Chr.
Chromosome 11 (human)
Chromosome 11 (human)
Genomic location for SERPING1
Genomic location for SERPING1
Band
bp
bp
Mouse
Chromosome 2 (mouse)

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