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Cobalamin-dependent enzyme
- Molecular classification
- Enzyme
01
Overview
Cobalamin-dependent enzymes are a class of enzymes that require cobalamin (vitamin B12) as a cofactor for their catalytic activity. Cobalamin is a complex organometallic molecule featuring a central cobalt atom, and it exists in several biologically active forms, most notably methylcobalamin (MeCbl) and adenosylcobalamin (AdoCbl). These enzymes play essential roles in various metabolic pathways across bacteria, fungi, animals, and humans. Key examples include methionine synthase and methylmalonyl-CoA mutase. Deficiencies or mutations affecting cobalamin-dependent enzyme function can lead to serious metabolic disorders.
02
Mechanism of action
Varies depending on the specific enzyme; includes methyl group transfer and radical-based rearrangement reactions.
03
Biological functions
Methyl group transferRadical-mediated rearrangementsAmino acid metabolismFatty acid catabolismEnvironmental detoxificationDNA synthesis
04
Disease associations
HomocystinuriaMethylmalonic acidemia
05
Safety considerations
Deficiencies can lead to serious metabolic disorders.Mutations affecting enzyme function can cause metabolic disorders.
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