De Flora A, Morelli A, Grasso M, Forteleoni G, Meloni T
Biomed Biochim Acta. 1987;46(2-3):S184-9.
Damaged RBC drawn from favic patients during acute hemolysis showed marked alterations in their two major proteolytic systems. Cytosolic procalpain (i.e., the proenzyme species of Ca2+-activated neutral proteinase, or calpain) had considerably lower activity than in matched RBC from asymptomatic G6PD-deficient subjects. The total RBC activity of the three acid endopeptidases that are normally membrane-bound was not reduced in favism, but its subcellular distribution was mostly cytosolic, suggesting quantitative release from membranes. Changes in procalpain activity are the result of both autoxidation of divicine and of the intracellular elevation of Ca2+ that is found in favism. Changes in acid endopeptidase activity are the consequence of perturbed Ca2+ homeostasis. Overall, the picture shows a marked impairment of the RBC proteolytic machinery that in turn may worsen cellular damage.
在急性溶血期间从蚕豆病患者体内提取的受损红细胞，其两个主要蛋白水解系统出现了显著变化。胞质中的前钙蛋白酶（即钙2+激活的中性蛋白酶或钙蛋白酶的酶原形式）的活性明显低于无症状的葡萄糖-6-磷酸脱氢酶（G6PD）缺乏受试者的匹配红细胞。通常与膜结合的三种酸性内肽酶的红细胞总活性在蚕豆病中并未降低，但其亚细胞分布主要在胞质中，这表明是从膜上定量释放出来的。前钙蛋白酶活性的变化是由于异胺基巴比妥酸的自氧化以及蚕豆病中出现的细胞内钙2+升高共同导致的。酸性内肽酶活性的变化是钙2+稳态受到干扰的结果。总体而言，情况显示红细胞蛋白水解机制存在明显受损，进而可能使细胞损伤恶化。
