Propionylcarnitine 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. References: Ghonimy A, Zhang DM, Farouk MH, Wang Q. The Impact of Carnitine on Dietary Fiber and Gut Bacteria Metabolism and Their Mutual Interaction in Monogastrics. Int J Mol Sci. 2018 Mar 28;19(4). pii: E1008. doi: 10.3390/ijms19041008. Review. PubMed PMID: 29597260; PubMed Central PMCID: PMC5979481.

纯度：≥98%

CAS：20064-19-1