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Molecular and metabolic aspects of lysosomal glycogen.
- Source :
-
Carbohydrate research [Carbohydr Res] 1992 Apr 06; Vol. 227, pp. 339-49. - Publication Year :
- 1992
-
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.
- Subjects :
- Acarbose
Animals
Blood Glucose analysis
Disease Models, Animal
Endopeptidase K
Glycoside Hydrolase Inhibitors
Lactates blood
Lysosomes drug effects
Phosphorylase Kinase deficiency
Pyruvates blood
Rats
Serine Endopeptidases metabolism
Trisaccharides pharmacology
Glycogen metabolism
Glycogen Storage Disease Type VII metabolism
Lysosomes metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0008-6215
- Volume :
- 227
- Database :
- MEDLINE
- Journal :
- Carbohydrate research
- Publication Type :
- Academic Journal
- Accession number :
- 1499032
- Full Text :
- https://doi.org/10.1016/0008-6215(92)85083-c