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Oxoglutarate Dehydrogenase Deficiency

Disease ID: disease_node_20524

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DbxrefGARD:617, MIM:203740, ORDO:31
SubclassofDOID_0050737, DOID_9252
Data SourceDOID
SynonymsOxoglutaric aciduria, alpha-ketoglutarate dehydrogenase deficiency
Doid Labeloxoglutarate dehydrogenase deficiency
Doid DescriptionAn amino acid metabolic disorder that is characterized by infantile and pediatric onset basal ganglia-associated movement disorders, hypotonia, developmental delays, ataxia, and seizures and that has_material_basis_in homozygous mutation in the oxoglutarate dehydrogenase gene (OGDH) on chromosome 7p13.
Existence Starts DuringHP_0003593
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20524
Doid IdDOID_0081326
LabelOxoglutarate Dehydrogenase Deficiency