Journal
IUBMB LIFE
Volume 74, Issue 7, Pages 592-617Publisher
WILEY
DOI: 10.1002/iub.2612
Keywords
coenzyme; coenzyme A; flavin adenine dinucleotide; mitochondria; mitochondrial carrier family SLC25; nicotinamide adenine dinucleotide; thiamine pyrophosphate
Categories
Funding
- Centre of Excellence on Comparative Genomics (CEGBA)
- Ministero dell'Istruzione, dell'Universita e della Ricerca (MIUR) [2020RRJP5L, 2017PAB8EM]
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Multiple mitochondrial matrix enzymes require cofactors for their action, which are transported by mitochondrial carriers. Mutations in mitochondrial cofactor carrier genes can compromise enzymatic activity and metabolic pathways.
Multiple mitochondrial matrix enzymes playing key roles in metabolism require cofactors for their action. Due to the high impermeability of the mitochondrial inner membrane, these cofactors need to be synthesized within the mitochondria or be imported, themselves or one of their precursors, into the organelles. Transporters belonging to the protein family of mitochondrial carriers have been identified to transport the coenzymes: thiamine pyrophosphate, coenzyme A, FAD and NAD(+), which are all structurally similar to nucleotides and derived from different B-vitamins. These mitochondrial cofactors bind more or less tightly to their enzymes and, after having been involved in a specific reaction step, are regenerated, spontaneously or by other enzymes, to return to their active form, ready for the next catalysis round. Disease-causing mutations in the mitochondrial cofactor carrier genes compromise not only the transport reaction but also the activity of all mitochondrial enzymes using that particular cofactor and the metabolic pathways in which the cofactor-dependent enzymes are involved. The mitochondrial transport, metabolism and diseases of the cofactors thiamine pyrophosphate, coenzyme A, FAD and NAD(+) are the focus of this review.
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