Abnormalities in gγ region of fibrinogen molecule and influences in assembly, secretion and function of intact fibrinogen
Author:
Publisher
Japanese Society on Thrombosis and Hemostasis
Subject
General Medicine
Reference12 articles.
1. 1) Asselta R, Duga S, Tenchini ML : The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost 4 : 2115-2129, 2006.
2. Fibrinogen Assembly and Secretion
3. 3) Terasawa F, Okumura N, Kitano K, Hayashida N, Shimosaka M, Okazaki M, Lord ST : Hypofibrinogenemia associated with a heterozygous missense mutation γ153Cys to Arg (Matsumoto IV) : In vitro expression demonstrates defective secretion of the variant fibrinogen. Blood 94 : 4122-4131, 1999.
4. 4) Okumura N, Terasawa F, Tanaka H, Hirota M, Ota H, Kitano K, Kiyosawa K, Lord ST : Analysis of fibrinogen γ-chain truncations shows the C-terminus, particularly γIle387, is essential for assembly and secretion of this multichain protein. Blood 99 : 3654-3660, 2002.
5. 5) Kani S, Terasawa F, Yamauchi K, Tozuka M, Okumura N : Analysis of fibrinogen variants at γ387Ile shows that the side chain of γ387 and the tertiary structure of the γC-terminal tail are important not only for assembly and secretion of fibrinogen but also for lateral aggregation of protofibrils and XIIIa-catalyzed γ-γ dimer formation. Blood 108 : 1887-1894, 2006.
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