Biomaterial Database

Leucine and glucose kinetics in glycogen storage disease type IIIa. 1997

O A Bodamer, and E Mayatepek, and J V Leonard

NMED unit, Institute of Child Health, London, UK.

UI	MeSH Term	Description	Entries
D007700	Kinetics	The rate dynamics in chemical or physical systems.
D007930	Leucine	An essential branched-chain amino acid important for hemoglobin formation.	L-Leucine,Leucine, L-Isomer,L-Isomer Leucine,Leucine, L Isomer
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
D008297	Male		Males
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005947	Glucose	A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement.	Dextrose,Anhydrous Dextrose,D-Glucose,Glucose Monohydrate,Glucose, (DL)-Isomer,Glucose, (alpha-D)-Isomer,Glucose, (beta-D)-Isomer,D Glucose,Dextrose, Anhydrous,Monohydrate, Glucose
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