Reactome | SLC25A19 transports ThDP from cytosol to mitochondrial matrix

SLC25A19 transports ThDP from cytosol to mitochondrial matrix

Stable Identifier

R-HSA-8875838

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial intermembrane space, mitochondrial inner membrane, mitochondrial matrix

ReviewStatus

5/5

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SLC25A19 transports ThDP from cytosol to mitochondrial matrix

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Mitochondrial thiamine pyrophosphate carrier (SLC25A19, DNC, MUP1) was originally thought to be a deoxyribonucleotide (DNC) carrier but has since been identified from enzyme kinetics, gene knockout studies and clinical samples from Amish Microcephaly (MCPHA) patients, to be a transporter of thiamine pyrophosphate (ThDP) into mitochondria (Kang & Samuels 2008). ThDP is a cofactor for the mitochondrial enzymes pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and branched chain amino acid dehydrogenase. The biochemical phenotype of MCPHA may be attributable to decreased activity of these enzymes (Siu et al. 2010).

Literature References

PubMed ID	Title	Journal	Year
18280798	The evidence that the DNC (SLC25A19) is not the mitochondrial deoxyribonucleotide carrier Samuels, DC, Kang, J	Mitochondrion	2008
20583149	Amish microcephaly: Long-term survival and biochemical characterization Rupar, CA, Prasad, AN, Prasad, C, Siu, VM, Ratko, S	Am. J. Med. Genet. A	2010