About The Medical Condition Brancher Deficiency
Find the definition of the medical term Brancher Deficiency. Brancher Deficiency defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Brancher Deficiency
| Brancher Deficiency Medical Condition |
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What's The Definition Of The Medical Condition Brancher Deficiency?
An autosomal recessive metabolic disorder due to a deficiency in expression of branching enzyme (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal glycogen with long outer branches. Clinical features are muscle hypotonia and cirrhosis. Death from liver disease usually occurs before age 2.
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