title: Assay of methylmalonyl CoA mutase with high-performance liquid chromatography.
abstract: An assay for methylmalonyl CoA mutase activity is described. Succinyl CoA produced in this method is separated from the substrate, methylmalonyl CoA, by reverse-phase high-performance liquid chromatography and is quantified. This method is useful to differentiate mutase apoenzyme deficiency (mut0, mut-) and the defect in deoxyadenosylcobalamin synthesis using fibroblasts cultured in high concentration of supplementary hydroxocobalamin. In methylmalonic acidemia, measurement of lymphocytes mutase activity offers therapeutical and prognostic informations.
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kikuchi
authors.forename: H
authors.initials: H
authors.lastname: Hanamizu
authors.forename: K
authors.initials: K
authors.lastname: Narisawa
authors.forename: K
authors.initials: K
authors.lastname: Tada
mesh_terms: D000214:Acyl Coenzyme A / Q000302:isolation & purification; D002851:Chromatography, High Pressure Liquid / Q000379:methods*; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007535:Isomerases / Q000302:isolation & purification*; D008214:Lymphocytes / Q000201:enzymology; D008765:Methylmalonyl-CoA Mutase / Q000302:isolation & purification* / Q000378:metabolism
chemical_list: D000214:Acyl Coenzyme A; C012046:succinyl-coenzyme A; D007535:Isomerases; D008765:Methylmalonyl-CoA Mutase