Immunoelectrophoretic studies on human small intestinal brush border proteins. The residual isomaltase in sucrose intolerant patients.
Small intestinal biopsies from three patients with sucrose intolerance (sucrase-isomaltase deficiency) were studied by means of immunoelectrophoresis and enzymatic assays. All patients lacked sucrase activity (less than 1 unit/g protein). One of the patients had a substantial isomaltase activity (7.8 unit/g protein). Immunoelectrophoresis revealed the presence of the isomaltase polypeptide of the sucrase-isomaltase in this patient. None of the biopsies showed any precipitate that might represent a modified inactive sucrase protein.
D000328:Adult; D002239:Carbohydrate Metabolism, Inborn Errors / Q000201:enzymology*; D002462:Cell Membrane / Q000201:enzymology*; D002675:Child, Preschool; D006026:Glycoside Hydrolases / Q000378:metabolism*; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D007583:Jejunum / Q000201:enzymology* / Q000648:ultrastructure; D008565:Membrane Proteins / Q000032:analysis; D008871:Microvilli / Q000201:enzymology*; D009834:Oligo-1,6-Glucosidase / Q000378:metabolism*; D013393:Sucrase / Q000378:metabolism; D013394:Sucrase-Isomaltase Complex / Q000378:metabolism; D013395:Sucrose / Q000378:metabolism*
D008565:Membrane Proteins; D013395:Sucrose; D006026:Glycoside Hydrolases; D013394:Sucrase-Isomaltase Complex; D009834:Oligo-1,6-Glucosidase; D013393:Sucrase