第八凝血因子

第八凝血因子（coagulation factor VIII）简称第八因子（factor VIII，FVIII），又称抗血友病因子A^[1][2]（antihemophilic factor A，AHF）^[3][4]，是一种凝血必须的蛋白质。人类的第八凝血因子由F8基因编码^[5][6]。F8基因的突变会导致X染色体连锁的凝血病甲型血友病。^[7]

第八凝血因子在肝血窦和全身的内皮细胞里产生。八因子以失活状态在血流里循环，与叫做血管性血友病因子（von Willebrand factor，vWF）的血浆载体结合，直到发生破环血管的损伤^[8]。八因子在损伤后被激活并从VWF中分离出来。这种活性蛋白（有时写作凝血因子VIIIa）会与另一种凝血因子IX发生相互作用（其机制尚不清楚）。这种相互作用会引发一系列额外的化学反应，最终形成血凝块^[8]。

延申阅读

• Gitschier J. The molecular basis of hemophilia A. Annals of the New York Academy of Sciences. 1991, 614 (1 Process in Va): 89–96. Bibcode:1991NYASA.614...89G. PMID 1902642. S2CID 26493612. doi:10.1111/j.1749-6632.1991.tb43694.x.
• White GC, Shoemaker CB. Factor VIII gene and hemophilia A. Blood. January 1989, 73 (1): 1–12. PMID 2491949.
• Antonarakis SE, Kazazian HH, Tuddenham EG. Molecular etiology of factor VIII deficiency in hemophilia A. Human Mutation. 1995, 5 (1): 1–22. PMID 7728145. S2CID 2346510. doi:10.1002/humu.1380050102 .
• Lenting PJ, van Mourik JA, Mertens K. The life cycle of coagulation factor VIII in view of its structure and function. Blood. December 1998, 92 (11): 3983–3996. PMID 9834200. doi:10.1182/blood.V92.11.3983.
• Saenko EL, Ananyeva N, Kouiavskaia D, Schwinn H, Josic D, Shima M, Hauser CA, Pipe S. Molecular defects in coagulation Factor VIII and their impact on Factor VIII function. Vox Sanguinis. August 2002, 83 (2): 89–96. PMID 12201837. doi:10.1046/j.1423-0410.2002.00183.x . hdl:2027.42/74861 .
• Lollar P. Molecular characterization of the immune response to factor VIII. Vox Sanguinis. 83. August 2002, 83 (Suppl 1): 403–408. PMID 12617176. S2CID 26050729. doi:10.1111/j.1423-0410.2002.tb05342.x.
• Fay PJ. Activation of factor VIII and mechanisms of cofactor action. Blood Reviews. March 2004, 18 (1): 1–15. PMID 14684146. doi:10.1016/S0268-960X(03)00025-0.
• Lavigne-Lissalde G, Schved JF, Granier C, Villard S. Anti-factor VIII antibodies: a 2005 update. Thrombosis and Haemostasis. October 2005, 94 (4): 760–769. PMID 16270627. S2CID 38533008. doi:10.1160/TH05-02-0118.
• Fang H, Wang L, Wang H. The protein structure and effect of factor VIII. Thrombosis Research. 2007, 119 (1): 1–13. PMID 16487577. doi:10.1016/j.thromres.2005.12.015.

外部链接

• GeneReviews/NCBI/NIH/UW entry on Hemophilia A
• The Coagulation Factor VIII Protein^[被篡夺]
• 医学主题词表（MeSH）：Factor+VIII
• PDB中UniProt可用的所有结构信息之概述：P00451 (Human Factor VIII) 在PDBe-KB（英语：PDBe-KB）。