Malkhassian Daniel, Sabir Sarah, Sharma Sandeep
Kaiser Permanente
Kansas University School of Medicine
Although discovered in 1944, Factor XIII's (FXIII) role as a deficiency disorder became evident in the 1960s upon identifying other clotting factors. Factor XIII, also referred to as fibrin stabilizing factor, plays a crucial role in the coagulation cascade by enhancing the stability of blood clot formation. The plasma form of Factor XIII is a protein heterodimer composed of A and B subunits expressed by bone marrow and mesenchymal lineage cells. Factor XIII functions as a transglutaminase, catalyzing peptide reactions responsible for cross-linking fibrin mesh. A deficiency can lead to life-threatening issues related to clot stability and hemostasis..
尽管因子 XIII(FXIII)于1944年被发现,但直到20世纪60年代发现其他凝血因子后,其作为一种缺乏症的作用才变得明显。因子 XIII,也被称为纤维蛋白稳定因子,通过增强血凝块形成的稳定性在凝血级联反应中起关键作用。因子 XIII 的血浆形式是一种蛋白质异二聚体,由骨髓和间充质谱系细胞表达的 A 和 B 亚基组成。因子 XIII 作为一种转谷氨酰胺酶发挥作用,催化负责交联纤维蛋白网的肽反应。缺乏该因子会导致与凝块稳定性和止血相关的危及生命的问题。