The effect of age on biochemical and morphological changes in the semitendinosus muscle of cattle with generalized glycogenosis type II.
Progressive changes in acid alpha-glucosidase activity, glycogen content and light microscopical and ultrastructural features in skeletal muscle of calves affected by generalized glycogenosis type II were assessed in biopsies from semitendinosus muscle of nine affected, twenty-six carrier and fifteen normal calves taken at varying times between birth and 17 months of age. Affected animals could be identified by using the PAS technique on paraffin and epon embedded material or by electron microscopy. However, estimation of acid alpha-glucosidase activity was required for precise diagnosis of generalized glycogenosis type II or to distinguish between normal and carrier animals. The glycogen content of the semitendinosus muscle of affected animals was approximately three times that in non-affected animals and although storage of glycogen reached a plateau soon after birth, the muscle fibre damage seen in very young calves increased with age. Morphological evidence of glycogen accumulation, both within the cytoplasm and within membrane bound structures, was present at birth. In some animals evidence of muscle fibre regeneration and damage was seen in the same sections.
D000367:Age Factors; D000818:Animals; D001706:Biopsy; D002417:Cattle; D005087:Glucan 1,4-alpha-Glucosidase / Q000172:deficiency; D006003:Glycogen / Q000378:metabolism; D006008:Glycogen Storage Disease / Q000473:pathology*; D006009:Glycogen Storage Disease Type II / Q000201:enzymology / Q000235:genetics / Q000473:pathology*; D006579:Heterozygote; D008854:Microscopy, Electron; D009132:Muscles / Q000473:pathology*; D000520:alpha-Glucosidases / Q000172:deficiency
D006003:Glycogen; D000520:alpha-Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase