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。