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Hmg-Coa Synthase 2 Deficiency

Disease ID: disease_node_20538

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DbxrefGARD:2712, MIM:605911, ORDO:35701
SubclassofDOID_0050737, DOID_9252
Data SourceDOID
Synonyms3-hydroxy-3-methylglutaryl-CoA synthase-2 deficiency
Doid LabelHMG-CoA synthase 2 deficiency
Doid DescriptionAn amino acid metabolic disorder that is characterized clinically by episodes of decompensation (often associated with gastroenteritis or fasting) that present with vomiting, lethargy, hepatomegaly, non ketotic hypoglycemia and, in rare cases, coma and that has_material_basis_in mutation in the HMGCS2 gene on chromosome 1p12.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20538
Doid IdDOID_0081168
LabelHmg-Coa Synthase 2 Deficiency