PMID: 7400341May 1, 1980

Fibrin cross-linking in congenital factor XIII deficiency

Journal of Clinical Pathology
F Rodeghiero, T Barbui

Abstract

Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine system), factor XIII molecular subunits, and the corresponding fibrin cross-linking pattern in seven homozygous patients with factor XIII deficiency. The results indicate that transamidase activity was present in all patients, and the range was 0.5-1.7%. The pattern of fibrin stabiisation showed an absence of cross-linking in two patients, the presence of gamma-gamma-dimers (traces) in four, and gamma-gamma-dimers plus incomplete alpha-polymers (traces) in one patient. In conclusion, the homozygous patients reported here were not completely devoid of functioning factor XIII.

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Citations

Oct 1, 1990·British Journal of Haematology·F Rodeghiero, A Tosetto
Jul 1, 1994·Protein Science : a Publication of the Protein Society·L C PedersenR E Stenkamp
Oct 1, 1989·American Journal of Reproductive Immunology : AJRI·J Kappelmayer, R Adány
Nov 19, 2013·Journal of the European Academy of Dermatology and Venereology : JEADV·D TöröcsikR Adány
Nov 8, 2014·Haemophilia : the Official Journal of the World Federation of Hemophilia·C AshleyD J Nugent
Dec 10, 2009·Blood·Kohji KasaharaA Ichinose
May 4, 1999·The Journal of Experimental Medicine·T NollH M Piper
Dec 30, 2018·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·L Lorand, Siiri E Iismaa
Aug 8, 2015·Molecular Genetics & Genomic Medicine·Anne ThomasJ Oldenburg
Jan 15, 2014·Haemophilia : the Official Journal of the World Federation of Hemophilia·V L SimhadriC Kimchi-Sarfaty
May 19, 2017·Haemophilia : the Official Journal of the World Federation of Hemophilia·V IvaškevičiusJ Oldenburg
Feb 1, 2003·Haemophilia : the Official Journal of the World Federation of Hemophilia·G PernodB Polack

Related Concepts

SDS-PAGE
Enzyme Activation
Factor XIII A-Chain
Factor Xiii Deficiency
Antithrombin I
Plasma Protein Binding Capacity
Gamma-Thrombin

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