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Combined Oxidative Phosphorylation Deficiency 12

Disease ID: disease_node_16734

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DbxrefGARD:13381, MIM:614924, ORDO:314051
SubclassofDOID_0050737, DOID_0060286
Data SourceDOID
SynonymsCOXPD12, LTBL, leukoencephalopathy with thalamus and brainstem involvement and high lactate, leukoencephalopathy-thalamus and brainstem anomalies-high lactate syndrome
Doid Labelcombined oxidative phosphorylation deficiency 12
Doid DescriptionA combined oxidative phosphorylation deficiency characterized by infantile onset of hypotonia and delayed psychomotor development or developmental regression that has_material_basis_in homozygous or compound heterozygous mutation in the EARS2 gene on chromosome 16p12.2.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_16734
Doid IdDOID_0111493
LabelCombined Oxidative Phosphorylation Deficiency 12