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Type 3 glycogenosis

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Definition

noun

A metabolic disorder that is caused by a deficiency of glycogen debranching enzyme (or amylo-1,6 glucosidase), resulting in the extra glycogen storage in cells, such as in liver and muscle


Supplement

Glycogenosis refers to a group of metabolic disorders due to the improper metabolism of glycogen. It involves certain enzymes that have become defective or deficient and therefore affected glycogen metabolism. The outcome is the excessive deposition of glycogen in cells. There are different types of glycogenosis and one of them is type 3 glycogenosis.

Type 3 glycogenosis is characterized by a deficiency in glycogen debranching enzyme (or amylo-1,6 glucosidase). A debranching enzyme is one that catalyzes the breakdown of glycogen. It is involved in mobilizing glucose from glycogen deposits in muscles and liver. Thus, when this enzyme is defective, this could lead to the manifestation of the symptoms associated with type 3 glycogenosis. The defective enzyme is caused by pathological mutation in the gene, AGL. Type 3 glycogenosis is inherited in an autosomal recessive pattern.

The common symptoms include neurological deterioration, myopathy, hepatomegaly, hypoglycemia, and hyperlipidemia. This condition causes retarded growth in German shepherd dogs at an early age.


Synonym(s):

  • Coris disease
  • debranching deficiency limit dextrinosis
  • glycogen storage disease type III (GSD III)
  • limit dextrinosis
  • forbes disease

See also: