title: Genetic relationship between lysosomal and lamellar body-specific alpha-glucosidases in human lung.
abstract: In lamellar bodies isolated from adult human lung at least two acid alpha-glucosidases are present: one similar to the lung lysosomal alpha-glucosidase, and another lamellar body-specific isoenzyme. In the present study we measured the activity of this lamellar body-specific alpha-glucosidase and of lysosomal alpha-glucosidase in a patient with an inherited deficiency of lysosomal alpha-glucosidase. The activity of the lamellar body-specific alpha-glucosidase was not affected in the patient, whereas the lysosomal alpha-glucosidase activity was strongly depressed. The results strongly suggest that the lysosomal alpha-glucosidase and the lamellar body-specific alpha-glucosidase are different gene products.
mesh_terms: D003208:Concanavalin A / Q000494:pharmacology; D006003:Glycogen / Q000494:pharmacology; D006008:Glycogen Storage Disease / Q000201:enzymology*; D006009:Glycogen Storage Disease Type II / Q000201:enzymology*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008168:Lung / Q000201:enzymology*; D008247:Lysosomes / Q000201:enzymology*; D000520:alpha-Glucosidases / Q000032:analysis* / Q000235:genetics / Q000276:immunology
chemical_list: D003208:Concanavalin A; D006003:Glycogen; D000520:alpha-Glucosidases