Maltase-glucoamylase and residual isomaltase in sucrose intolerant patients

J Pediatr Gastroenterol Nutr. May-Jun 1986;5(3):365-71. doi: 10.1097/00005176-198605000-00005.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Carbohydrate Metabolism, Inborn Errors / enzymology*
  • Child
  • Child, Preschool
  • Glucan 1,4-alpha-Glucosidase / metabolism*
  • Glucosidases / metabolism*
  • Glycoside Hydrolases / metabolism*
  • Humans
  • Immunoelectrophoresis
  • Immunoelectrophoresis, Two-Dimensional
  • Immunoenzyme Techniques
  • Jejunum / enzymology
  • Multienzyme Complexes / deficiency*
  • Oligo-1,6-Glucosidase / metabolism*
  • Sucrase / metabolism
  • Sucrase-Isomaltase Complex / deficiency*
  • Sucrose / metabolism*

Substances

  • Multienzyme Complexes
  • Sucrose
  • Glucosidases
  • Glycoside Hydrolases
  • Sucrase-Isomaltase Complex
  • Oligo-1,6-Glucosidase
  • Glucan 1,4-alpha-Glucosidase
  • Sucrase