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Fibrinogen Kurashiki (γ G268E) Fibrin Network Structure

Published online by Cambridge University Press:  02 July 2020

J. P. DiOrio ,
M. W. Mosesson  and
M. Matsuda
J. P. DiOrio
Affiliation:
Baxter International, Round Lake, IL60073
M. W. Mosesson
Affiliation:
Sinai Samaritan Med Ctr, Univ Wise Med Schl-Milw Clin Campus, Milwaukee, WI53201
M. Matsuda
Affiliation:
Jichi Med Schl, Tochigi-Ken 329-04, Japan
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Extract

Upon thrombin-catalyzed proteolytic conversion of fibrinogen to fibrin, fibrinopeptide A release exposes an N-terminal polymerization site (‘EA’) in the central E domain that interacts with a constitutive complementary site in each outer D domain (‘Da’) to drive the molecular assembly process by end-to-middle domain non-covalent ‘Da:EA’ interactions. End-to-end aligned doublestranded fibrils are formed in coordination with another recently described constitutive self-association site (termed ‘D:D’)that is situated at the outer end of each D domain. The D:D site contributes to end-to-end molecular alignment of fibrin molecules within each fibril strand. Concomitant branching and lateral fibril associations to form thick fibers, lead to formation of the mature fibrin clot.

Fibrinogen ‘Kurashiki’ is a unique congenital dysfibrinogenemia that was identified in a homozygous father and his heterozygous son. The abnormality involves a γ chain amino acid mutation (γ G268E) and is characterized by delayed and abnormal fibrin polymerization.

Type
Biological Ultrastructure/Microbiology
Information
Microscopy and Microanalysis , Volume 4 , Issue S2: Proceedings: Microscopy & Microanalysis '98, Microscopy Society of America 56th Annual Meeting, Microbeam Analysis Society 32nd Annual Meeting, Atlanta, Georgia July 12-16, 1998 , July 1998 , pp. 1160 - 1161
Copyright
Copyright © Microscopy Society of America

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