Dubs R, Gitzelmann R, Steinmann B, Lindenmann J
Helv Paediatr Acta. 1975 May;30(1):89-102.
By immunofluorescence microscopy using a specific antibody to the active rabbit sucrase-isomaltase complex, a catalytically inactive sucrase antigen was discovered on the small intestinal mucosa of young rabbits still lacking sucrase activity. In adult rabbits, the same antigen was demonstrated on enterocytes of mucosal crypts devoid of sucrase. Catalytically inactive antigen was isolated by means of immobilized antibody to active sucrase, and it was compared with the active sucrase-isomaltase complex. From structural similarities between the 2 proteins and from the fact that active sucrase succeeds the inactive antigen in both the maturing and the mature rabbit, it is concluded that the inactive antigen is the enzyme precursor. In some patients with hereditary sucrase-isomaltase deficiency, abnormal persistence of sucrase precurosr due to a faulty activation mechanism may be the underlying defect.
通过使用针对活性兔蔗糖酶 - 异麦芽糖酶复合物的特异性抗体进行免疫荧光显微镜检查,在仍缺乏蔗糖酶活性的幼兔小肠黏膜上发现了一种催化无活性的蔗糖酶抗原。在成年兔中,在缺乏蔗糖酶的黏膜隐窝的肠细胞上也证实了相同的抗原。通过针对活性蔗糖酶的固定化抗体分离出催化无活性的抗原,并将其与活性蔗糖酶 - 异麦芽糖酶复合物进行比较。从这两种蛋白质的结构相似性以及活性蔗糖酶在幼兔成熟和成年过程中接替无活性抗原这一事实可以得出结论,无活性抗原是酶前体。在一些遗传性蔗糖酶 - 异麦芽糖酶缺乏症患者中,由于激活机制错误导致蔗糖酶前体异常持续存在可能是潜在缺陷。