Skovbjerg H, Krasilnikoff P A
J Pediatr Gastroenterol Nutr. 1986 May-Jun;5(3):365-71. doi: 10.1097/00005176-198605000-00005.
Small intestinal biopsies from nine patients with sucrase-isomaltase deficiency (sucrose-intolerance) were analyzed. All patients lacked sucrase activity and three patients had a residual isomaltase activity and a corresponding isomaltase precipitate following immunoelectrophoresis. By polyacrylamide gel electrophoresis in sodium dodecyl sulfate followed by immunoblotting the residual isomaltase appeared as a single polypeptide with molecular weight of approximately 145,000. Maltase-glucoamylase in the biopsies was specifically quantitated by crossed immunoelectrophoresis. One of the patients had an almost total deficiency of maltase-glucoamylase in the biopsy, three patients had a normal amount of maltase-glucoamylase, and five patients constituted an intermediary group. These results indicate that some of the sucrase-isomaltase deficient patients also have a more or less pronounced deficiency of maltase-glucoamylase. The patients constitute an even more heterogeneous group than earlier suggested and should be classified by the amount not only of sucrase and isomaltase but also of maltase-glucoamylase.
对9例蔗糖酶-异麦芽糖酶缺乏症（蔗糖不耐受）患者的小肠活检组织进行了分析。所有患者均缺乏蔗糖酶活性，3例患者有残余异麦芽糖酶活性，免疫电泳后有相应的异麦芽糖酶沉淀。通过十二烷基硫酸钠聚丙烯酰胺凝胶电泳及免疫印迹法，残余异麦芽糖酶表现为一条分子量约为145,000的单一多肽。活检组织中的麦芽糖酶-葡糖淀粉酶通过交叉免疫电泳进行特异性定量。其中1例患者活检组织中麦芽糖酶-葡糖淀粉酶几乎完全缺乏，3例患者麦芽糖酶-葡糖淀粉酶含量正常，5例患者构成中间组。这些结果表明，一些蔗糖酶-异麦芽糖酶缺乏的患者也或多或少存在明显的麦芽糖酶-葡糖淀粉酶缺乏。这些患者构成的异质性群体比之前认为的更大，不仅应根据蔗糖酶和异麦芽糖酶的量，还应根据麦芽糖酶-葡糖淀粉酶的量进行分类。
