_:vb50949735 . _:vb50949735 "An amino acid metabolic disorder characterized by inability to process isoleucine and ketones, has_symptom recurrent ketoacidotic attacks in infancy marked by vomitting, lethargy, dehydration, and seizures, and has_material_basis_in mutation in the ACAT1 gene of chromosome 11q22.3 responsible for producing the ACAT1 enzyme in mitochondria, which processes isoleucine and ketones."^^ . _:vb50949735 "url:https://ghr.nlm.nih.gov/condition/beta-ketothiolase-deficiency#statistics"^^ . _:vb50949735 . _:vb50949735 . _:vb50949735 . _:vb50949735 .