Proline 36 of the Factor XIII Activation Peptide Plays a Crucial Role in Substrate Recognition and Zymogen Activation.

ABSTRACT: The activation peptide of blood coagulation factor XIII (AP-FXIII) has important functions in stabilizing the FXIII-A₂ dimer and regulating FXIII activation. Contributions of many of its 37 amino acids to these functions have been described. However, the role of proline 36, which is adjacent to the thrombin cleavage site at Arg37, has not yet been studied in detail. We approached this question when we came across a patient with congenital FXIII deficiency in whom we detected a novel Pro36Ser mutation. We expressed the mutant FXIII-A Pro36Ser protein in Chinese hamster ovary cells and found that this mutation does not influence FXIII-A expression but significantly inhibits proteolytic activation by thrombin. The enzymatic transglutaminase activity is not affected as it can be ind

SUBMITTER: Li B 

PROVIDER: S-EPMC6716785 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature