第八凝血因子

第八凝血因子（英語：Coagulation factor VIII, Factor VIII, FVIII, anti-hemophilic factor (AHF)) 是一種凝血必須的蛋白質。人類的第八凝血因子由F8基因編碼^[1][2]。F8基因的突變會導致X染色體連鎖的凝血病甲型血友病。^[3]

第八凝血因子在肝血竇和全身的內皮細胞里產生。八因子以失活狀態在血流里循環，與叫做類血友病因子(英語：von Willebrand factor, VWF)的血漿載體結合，直到發生破環血管的損傷。^[4]八因子在損傷後被激活並從VWF中分離出來。這種活性蛋白（有時寫作凝血因子VIIIa）會與另一種凝血因子IX發生相互作用（其機制尚不清楚）。這種相互作用會引發一系列額外的化學反應，最終形成血凝塊。^[4]

延申閱讀

• 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.

外部連結

• GeneReviews/NCBI/NIH/UW entry on Hemophilia A
• The Coagulation Factor VIII Protein^[usurped]
• 醫學主題詞表（MeSH）：Factor+VIII
• PDB中UniProt可用的所有結構信息之概述：P00451 (Human Factor VIII) 在PDBe-KB（英語：PDBe-KB）。