Molecular and metabolic aspects of lysosomal glycogen

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.