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Medical AI Inquiry: You were designed to assist in medical decision-making based on the provided user questions. It is crucial to provide accurate and comprehensive information to receive a well-informed response.
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Andersen disease is due to lack of: March 2007
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The correct answer is  Ans. A: Branching enzymeAndersen disease/Glycogen storage disease type IV is a very rare hereditary metabolic disorder.Synonyms:Glycogenosis type IV,Glycogen Branching Enzyme Deficiency (GBED),Polyglucosan body disease.AmylopectinosisIt is a result of the absence of the glycogen branching enzyme amylo-1,4-1,6 transglucosidase, which is critical in the production of glycogen.This leads to very long unbranched glucose chains being stored in glycogen (known as amylopectin) which have a low solubility leading to glycogen precipitation in the liver.These deposits subsequently build up in the body tissue, especially the hea and liver.The end result is liver failure and eventual death occurring in the first year of life.