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先天性蔗糖酶-异麦芽糖酶缺乏症肠道黏膜的亚细胞分级分离研究

Subcellular fractionation studies of the intestinal mucosa in congenital sucrase--isomaltase deficiency.

作者信息

Cooper B T, Candy D C, Harries J T, Peters T J

出版信息

Clin Sci (Lond). 1979 Aug;57(2):181-5. doi: 10.1042/cs0570181.

Abstract
  1. Jejunal biopsy specimens from three children with congenital sucrase-isomaltase deficiency were assayed for disaccharidase activity and were subjected to analytical subcellular fractionation with enzymic microanalysis. 2. By use of the highly sensitive fluorigenic modification of the disaccharidase assay, brush-border sucrase and isomaltase activities were depressed but nevertheless detectable in each child. 3. Apart from the expected decrease in brush-border alpha-glucosidase activity, the other enterocyte marker-enzyme activities were normal. 4. There were no abnormalities in the enterocytes of any child on analytical subcellular fractionation or on electron microsocopy.
摘要
  1. 对三名患有先天性蔗糖酶-异麦芽糖酶缺乏症的儿童的空肠活检标本进行双糖酶活性测定,并通过酶微分析进行亚细胞分级分离分析。2. 通过使用双糖酶测定的高灵敏度荧光法,每个儿童的刷状缘蔗糖酶和异麦芽糖酶活性均降低,但仍可检测到。3. 除了刷状缘α-葡萄糖苷酶活性预期降低外,其他肠上皮细胞标记酶活性均正常。4. 在亚细胞分级分离分析或电子显微镜检查中,任何儿童的肠上皮细胞均无异常。

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