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Home » MUSCLE PATHOLOGY » Glycogen Storage Disease (Glycogenosis)

Friday, September 7, 2012

Glycogen Storage Disease (Glycogenosis)

Progressive muscular weakness and inability to rise properly may be seen in animals with glycogen storage diseases. To date, 5 of the 8 types of glycogen storage diseases characterized in humans have been identified in animals (types I, II, III, VII, and VIII). Affected species include cattle, sheep, dogs, cats, horses, Japanese quail, rats, and mice. Type II glycogenosis in Shorthorn and Brahman cattle has been well documented and is inherited as an autosomal recessive disorder. Affected cattle develop muscular weakness and die at 9-16 mo of age, often with accompanying cardiomegaly and congestive heart failure. Morphologic and biochemical study reveals extensive intralysosomal and cytoplasmic glycogen deposits. Corriedale sheep and Lapland dogs also develop type II glycogenosis.
Myophosphorylase deficiency (type V glycogenosis) is an autosomal recessive disorder in Charolais cattle. Affected cattle show exercise intolerance and may have increased serum activities of skeletal muscle-origin enzymes.
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