5'-deoxyadenosylcobalamin

(redirected from 5-deoxyadenosylcobalamin)

5'-de·ox·y·a·den·o·syl·co·bal·a·min

(dē-oks'ē-ă-den'ō-sil-kō-bal'ă-min),
An active coenzyme form of vitamin B12; required in the conversion of methylmalonyl-CoA to succinyl-CoA. A deficiency of 5'-deoxyadenosylcobalamin produces methylmalonic acidemia.
References in periodicals archive ?
5-deoxyadenosylcobalamin (AdoCbl) is required by methylmalonyl-CoA mutase, which catalyzes the conversion of L- methylmalonyl-CoA to succinyl-CoA.
The generation of methionine from homocysteine requires cytoplasmic methylcobalamin and the conversion of methyl-malonyl-coenzyme A to succinyl-coenzyme A requires mitochondrial 5-deoxyadenosylcobalamin [45].
The biologically active coenzyme forms of vitamin B12 that play a role in human metabolism are methylcobalamin and 5-deoxyadenosylcobalamin (commonly known as adenosylcobalamin).
The basis of an elevated MMA concentration as a sensitive functional indicator of vitamin [B.sub.12] deficiency is that 5-deoxyadenosylcobalamin is an essential cofactor for the conversion of methylmalonyl-CoA to succinyl-CoA.
MMA is caused by a defect in the conversion of L-methylmalonyl-CoA to succinyl-CoA, catalyzed by the enzyme methylmalonylCoA (MMCoA) mutase (EC.5.4.99.2), which requires 5-deoxyadenosylcobalamin (AdoCbl) as a cofactor.