A glycogen storage disease in rats. Morphological and biochemical investigations.
Liver and heart from a substrain of the NZR/Gd rat in which there is an inherited deficiency of liver phosphorylase b kinase was examined by light and electron microscopy and compared to material from a related, but normal substrain. Hepatic tissue differed markedly from that of control animals. Hepatocytes contained more than twice as much free glycogen and visible lipid. Glycogen particles had an abnormal appearance and some glycogen was sequestered within large, membrane-bound vesicles. Hepatocyte lysosomes were increased by a third and mean cell volume by more than half. Lobular architecture was distorted by the presence of enlarged, irregularly-shaped hepatocytes. Free glycogen was present in the space of Disse and sinusoids and within lysosomes in Kupffer cells. There were increased amounts of collagen in the space of Disse. The changes resemble those described in human glycogen storage disease IXa. A study of hepatic tissue from fasted rats showed that affected animals have an impaired ability to mobilise their liver glycogen stores. An increase in visible lipid also occurred in affected, fasted animals. Cardiac tissue appeared to be normal.
D000818:Animals; D003094:Collagen / Q000032:analysis; D005215:Fasting; D005260:Female; D006003:Glycogen / Q000032:analysis; D006008:Glycogen Storage Disease / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007728:Kupffer Cells / Q000648:ultrastructure; D008099:Liver / Q000378:metabolism / Q000648:ultrastructure*; D008247:Lysosomes / Q000648:ultrastructure; D008854:Microscopy, Electron; D009206:Myocardium / Q000648:ultrastructure; D006005:Phosphorylases / Q000172:deficiency*; D051381:Rats; D011919:Rats, Inbred Strains
D006003:Glycogen; D003094:Collagen; D006005:Phosphorylases