CAS #
20064-19-1
Concentration
98.00%
Description
Propinol-L-Carnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency, together with Methylmalonic acid. MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA.
Keywords
(2R)-3-Carboxy-N,N,N-trimethyl-2-(1-oxopropoxy)-1-propanaminium inner salt, L-Carnitine propionyl ester, C3-Carnitine, Propanoyl-L-carnitine