Multiple metabolic enzymes using thiamine pyrophosphate, pyridoxal 5'-phosphate, and cobalamin as cofactors

This target refers to a diverse group of metabolic enzymes that require specific B-vitamin derivatives—thiamine pyrophosphate (TPP), pyridoxal 5'-phosphate (PLP), and cobalamin—as essential cofactors for catalytic activity (Abcam, PMC). TPP-dependent enzymes, such as the pyruvate dehydrogenase complex and transketolase, are critical for carbohydrate metabolism and energy production via the TCA cycle (PMC, NIH). PLP-dependent enzymes are primarily involved in amino acid metabolism and the synthesis of neurotransmitters like dopamine and GABA (PMC, ResearchGate). Cobalamin-dependent enzymes, including methionine synthase and methylmalonyl-CoA mutase, play vital roles in DNA synthesis, methylation, and fatty acid metabolism (Abcam, NIH). Deficiencies or genetic mutations affecting these enzymes lead to severe metabolic disorders, including Wernicke-Korsakoff syndrome, homocystinuria, and megaloblastic anemia (PMC, PNAS). Therapeutic intervention typically involves high-dose cofactor supplementation to restore enzymatic function or manage metabolic flux (PNAS).

Other names

B-vitamin dependent enzymesTPP-dependent enzymesPLP-dependent enzymesCobalamin-dependent enzymesPyruvate dehydrogenase complexAlpha-ketoglutarate dehydrogenase complexBranched-chain alpha-keto acid dehydrogenase complexTransketolaseMethionine synthaseMethylmalonyl-CoA mutaseAromatic L-amino acid decarboxylase

Cofactor supplementation to restore holoenzyme activity (PNAS); enzymatic inhibition by vitamin antagonists (PMC).