Term Name:	3-hydroxyisobutryl-CoA hydrolase deficiency
Synonyms:	HIBCH deficiency, Methacrylic aciduria, Valine metabolic defect
Definition:	An amino acid metabolic disorder characterized by severely delayed psychomotor development, neurodegeneration, increased lactic acid, and brain lesions in the basal ganglia that has_material_basis_in homozygous or compound heterozygous mutation in the HIBCH gene on chromosome 2q32.
Ontology:	Human Disease [DOID:0060949]   ( DOID:0060949 )