glycogenosis

Pl. glycogenoses; any genetically determined disorder of glycogen metabolism, marked by abnormal storage of glycogen in the tissues of the body. See also glycogen storage disease.

• bovine generalized g. — an inherited glycogen storage disease of Shorthorn and Brahman cattle resembling glycogenosis type II (Pompe's disease) of humans, caused by a deficiency of α-1,4-glucosidase. Widespread accumulation of glycogen occurs in the nervous system and muscles, leading to poor growth, incoordination, muscle weakness and eventually recumbency. There is also cardiomyopathy and often left-sided heart failure. Onset is at 2 to 3 months of age with death at 3 to 5 months. A late onset form with a short clinical course is described in 8 to 9 month old Brahman cattle.
• g. type I — in humans, a deficiency of the hepatic enzyme glucose-6-phosphatase resulting in liver and kidney involvement, with hepatomegaly, hypoglycemia, hyperuricemia and gout. A similar condition has been observed in young dogs with hypoglycemia that does not respond to glucagon. Called also Gierke's disease or von Gierke's disease.
• g. type II — see bovine generalized glycogenosis (above). Also reported in sheep, cats, dogs and quail. Called also Pompe's disease.
• g. type III — an inherited deficiency of amylo-1,6-glucosidase causing neurological deterioration, hepatomegaly and retarded growth in German shepherd dogs from an early age. Called also Cori's disease, Forbes’ disease, limit dextrinosis.
• g. type IV — an inherited deficiency of glycogen branching enzyme activity resulting in storage of abnormal glycogen, especially in the liver and spleen. Reported in Norwegian forest cats. Called also Andersen disease, amylopectinosis.
• g. type V — an inherited deficiency of myophosphorylase which results in muscle cramping with exercise. Reported in Charolais calves. Called also McArdle disease.
• g. type VII — see phosphofructokinase 1 deficiency.