Biomaterial Database

[Disorder of liver glycogen metabolism in glycogenosis 3]. 1976

I V Chibisov, and I A Popova, and E L Rozenfel'd

UI	MeSH Term	Description	Entries
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008112	Liver Glycogen	Glycogen stored in the liver. (Dorland, 28th ed)	Hepatic Glycogen,Glycogen, Hepatic,Glycogen, Liver
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D006008	Glycogen Storage Disease	A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement.	Glycogenosis,Disease, Glycogen Storage,Diseases, Glycogen Storage,Glycogen Storage Diseases,Glycogenoses,Storage Disease, Glycogen,Storage Diseases, Glycogen
D006010	Glycogen Storage Disease Type III	An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.	Cori's Disease,Debrancher Deficiency,Forbes Disease,Glycogen Debranching Enzyme Deficiency,Glycogenosis 3,Limit Dextrinosis,Amylo-1,6-Glucosidase Deficiency,Cori Disease,Deficiency, Debrancher,Glycogen Debrancher Deficiency,Glycogen Storage Disease III,Glycogen Storage Disease Type 3,Amylo 1,6 Glucosidase Deficiency,Amylo-1,6-Glucosidase Deficiencies,Coris Disease,Debrancher Deficiencies,Debrancher Deficiencies, Glycogen,Debrancher Deficiency, Glycogen,Deficiencies, Amylo-1,6-Glucosidase,Deficiencies, Debrancher,Deficiencies, Glycogen Debrancher,Deficiency, Amylo-1,6-Glucosidase,Deficiency, Glycogen Debrancher,Dextrinoses, Limit,Dextrinosis, Limit,Disease, Cori,Disease, Cori's,Disease, Forbes,Glycogen Debrancher Deficiencies,Glycogenosis 3s,Limit Dextrinoses
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man