Fuhrer G, Gallimore M J, Heller W, Hoffmeister H E
Kreiskrankenhaus Reutlingen, Federal Republic of Germany.
Blut. 1990 Nov;61(5):258-66. doi: 10.1007/BF01732874.
The plasma protein FXII (Hageman factor) has been shown to be linked with the plasma defence systems of coagulation, fibrinolysis, kallikrein-kinin and complement. It can be activated by surface contact activation and in solution. Surface contact activation is a complex phenomenon involving negatively charged surfaces, FXII, high molecular weight kininogen and plasma kallikrein. Fluid-phase activation can be effected by a variety of serine proteases. In both types of activation the FXII zymogen is converted to active enzymes. FXII levels in plasma are low or undetectable in both inherited deficiencies and in a variety of clinical conditions. FXII levels can also be elevated in some clinical conditions. Although discovered as a clotting protein FXII appears to play an important role in the kallikrein-kinin and fibrinolytic systems and also has effects on cells. Recent studies suggest that therapeutic blockade of activation of FXII can be of benefit in certain clinical conditions.
血浆蛋白FXII(哈格曼因子)已被证明与凝血、纤维蛋白溶解、激肽释放酶-激肽和补体等血浆防御系统有关。它可通过表面接触激活和在溶液中被激活。表面接触激活是一种复杂的现象,涉及带负电荷的表面、FXII、高分子量激肽原和血浆激肽释放酶。液相激活可由多种丝氨酸蛋白酶实现。在这两种激活类型中,FXII酶原都转化为活性酶。在遗传性缺陷和多种临床病症中,血浆中的FXII水平较低或检测不到。在某些临床病症中,FXII水平也可能升高。尽管FXII最初是作为一种凝血蛋白被发现的,但它似乎在激肽释放酶-激肽和纤维蛋白溶解系统中发挥重要作用,并且对细胞也有影响。最近的研究表明,在某些临床病症中,对FXII激活进行治疗性阻断可能有益。