An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (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.

Descriptor ID	D006011
MeSH Number(s)	C16.320.565.202.449.540 C18.452.648.202.449.540
Concept/Terms	Glycogen Storage Disease Type IV Glycogen Storage Disease Type IV Andersen Disease Disease, Andersen Andersen's Disease Andersens Disease Disease, Andersen's Brancher Deficiency Brancher Deficiencies Deficiencies, Brancher Deficiency, Brancher Glycogen Branching Enzyme Deficiency Glycogen Storage Disease Type 4 Glycogenosis 4 Glycogenosis 4s Glycogenosis IV Glycogenosis IVs Type IV Glycogenosis Glycogenoses, Type IV Glycogenosis, Type IV Type IV Glycogenoses Amylopectinosis Amylopectinoses Gbe1 Deficiency Deficiencies, Gbe1 Deficiency, Gbe1 Gbe1 Deficiencies

Below are MeSH descriptors whose meaning is more general than "Glycogen Storage Disease Type IV".

Below are MeSH descriptors whose meaning is related to "Glycogen Storage Disease Type IV".

Below are MeSH descriptors whose meaning is more specific than "Glycogen Storage Disease Type IV".