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Childhood-Onset Dystonia With Optic Atrophy And Basal Ganglia Abnormalities

Disease ID: disease_node_14355

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DbxrefGARD:13488, MIM:617282, ORDO:508093
SubclassofDOID_543, DOID_0050737
Data SourceDOID
SynonymsDYSTONIA 29, CHILDHOOD-ONSET, DYTOABG, MECR-related neurologic disorder, MEPAN syndrome, Mitochondrial Enoyl CoA Reductase Protein-Associated Neurodegeneration
Doid Labelchildhood-onset dystonia with optic atrophy and basal ganglia abnormalities
Doid DescriptionA dystonia that is characterized by characterized by onset of involuntary movements in the first decade of life and that has_material_basis_in homozygous or compound heterozygous mutation in the MECR gene on chromosome 1p35. Optic atrophy develops around the same time or slightly later.
Existence Starts DuringHP_0011463
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_14355
Doid IdDOID_0081419
LabelChildhood-Onset Dystonia With Optic Atrophy And Basal Ganglia Abnormalities