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Glycogen Storage Disease Type Iii

Disease ID: disease_node_3524

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DbxrefICD10CM:E74.03, MESH:D006010, MIM:232400, NCI:C84736, SNOMEDCT_US_2023_03_01:66937008, UMLS_CUI:C0017922
SubclassofDOID_2747, DOID_0050737
Data SourceDOID, MESH
SynonymsGlycogen storage disease 3, Glycogen storage disease, type III, amylo 1,6 glucosidase deficiency, deficiency of debranching enzyme, deficiency of dextrin
Mesh IdD006010
Mesh LabelGlycogen Storage Disease Type III
Mesh SubclassofD006008
Doid Labelglycogen storage disease III
Doid DescriptionA glycogen storage disease that is characterized by an accumulation of abnormal glycogen with short outer chains and that has_material_basis_in homozygous or compound heterozygous mutation in the AGL gene, which encodes the glycogen debrancher enzyme, on chromosome 1p21. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_3524
Doid IdDOID_2748
LabelGlycogen Storage Disease Type Iii