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What is Andersen disease is due to lack of: March 2007 and explain in detail?
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.