SLC25A19 transports ThDP from cytosol to mitochondrial matrix

Stable Identifier
Reaction [transition]
Homo sapiens
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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
Catalyst Activity

thiamine pyrophosphate transmembrane transporter activity of SLC25A19 [mitochondrial inner membrane]

Orthologous Events
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