Franci C,Marder VJ. Increased resistance to plasmic degradation of fibrin with highly crosslinked a-polymer chains formed at high factor XIII concentrations[J].Blood.1988,71:1361. 薛毅
Factor XIII or fibrin stabilizing factor is an enzyme (EC 220.127.116.11) of the blood coagulation system that crosslinks fibrin. 什么是'因子十三缺陷-罕见的疾病所造成的凝血因子缺乏症'?
The 2 mutations of the factor XIIIA cause the instability, degradation, and rapid disappearance of FXIIIA in cytoplasm, thus resulting in hereditary factor XIII deficiency. 两种FXIIIA基因突变导致FXIIIA在细胞内不稳定 ,迅速被降解 ,从而FXIIIA蛋白量减少和活性丧失。