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.
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This organic acid buildup is caused by one of three mechanisms: genetic defect or absence of the enzyme methylmalonic acid coenzyme A mutase (mut), defect in the synthesis and transport of mut's cofactor 5'-deoxyadenosylcobalamin, and defect in the mitochondrial transport of cobalamin.
The major forms are hydroxocobalamin or aquocobalamin (OHCbl) and the 2 coenzyme forms of Cbl, 5'-deoxyadenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl) (1, 13).
It also seems worth pondering why 5'-deoxyadenosylcobalamin makes up more of the cobalamin in holo-TC than in holo-HC (7).