propionyl-CoA

(redirected from Propionyl CoA)

pro·pi·o·nyl-CoA

(prō'pē-ō-nil),
The coenzyme A thioester derivative of propionic acid; an intermediate in the degradation of l-valine, l-isoleucine, l-threonine, l-methionine, and odd-chain fatty acids; a precursor for the synthesis of odd-chain fatty acids; it accumulates in individuals with a deficiency of propionyl-CoA carboxylase.
References in periodicals archive ?
LDHA, lactate dehydrogenase A; LDHB, lactate dehydrogenase B; PDHA1, pyruvate dehydrogenase (lipoamide) alpha 1; DLAT, dihydrolipoamide S-cetyltransferase; DLD, dihydrolipoamide dehydrogenase; GK, glycerol kinase; GPD1, glycerol-3-phosphate dehydrogenase 1; GPD2, glycerol -3-phosphate dehydrogenase 2; ACSS3, acyl CoA synthetase short chain family member 3; PCCA, propionyl CoA carboxylase alpha; PCCB, propionyl CoA carboxylase beta; MCEE, methylmalonyl CoA epimerase; MUT, methylmalonyl CoA mutase.
In addition, CRTAase was also found to utilize propionyl CoA as the propionyl group donor (unpublished data).
The hyperammonemia observed in methylmalonic acidemia is thought to arise because accumulated propionyl CoA interferes with formation of N-acetylglutamate, an obligatory activator of carbamyl phosphate synthase, the initial step in urea synthesis (4).