title: Molecular and metabolic aspects of lysosomal glycogen.
abstract: The high molecular weight glycogen associated with the lysosomal compartment in glycogen storage disease type VIII is more resistant to degradation by proteinase than normal glycogen. The assembly of large glycogen particles on disulphide-linked protein backbones has been confirmed and the disulphide-reducing nature of the lysosome appears to confer an advantage in the amylolytic degradation of glycogen. Experiments utilising acarbose, a lysosomal (1----4)-alpha-D-glucosidase inhibitor, show that some blood glucose could arise in normal mammals from extra-hepatic tissue, by degradation of the glycogen in the lysosomal compartment.
authors.affiliation: Department of Biochemistry, University of Auckland, New Zealand.
authors.forename: R
authors.initials: R
authors.lastname: Geddes
authors.forename: P
authors.initials: P
authors.lastname: Jeyarathan
authors.forename: J A
authors.initials: JA
authors.lastname: Taylor
mesh_terms: D020909:Acarbose; D000818:Animals; D001786:Blood Glucose / Q000032:analysis; D004195:Disease Models, Animal; D019286:Endopeptidase K; D006003:Glycogen / Q000378:metabolism*; D006014:Glycogen Storage Disease Type VII / Q000378:metabolism*; D065089:Glycoside Hydrolase Inhibitors; D007773:Lactates / Q000097:blood; D008247:Lysosomes / Q000187:drug effects / Q000378:metabolism*; D010764:Phosphorylase Kinase / Q000172:deficiency; D011773:Pyruvates / Q000097:blood; D051381:Rats; D012697:Serine Endopeptidases / Q000378:metabolism; D014312:Trisaccharides / Q000494:pharmacology
chemical_list: D001786:Blood Glucose; D065089:Glycoside Hydrolase Inhibitors; D007773:Lactates; D011773:Pyruvates; D014312:Trisaccharides; D006003:Glycogen; D010764:Phosphorylase Kinase; D012697:Serine Endopeptidases; D019286:Endopeptidase K; D020909:Acarbose