Reaction: SLC25A19 transports ThDP from cytosol to mitochondrial matrix
- in pathway: Vitamin B1 (thiamin) metabolism
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).
Reaction - small molecule participants:
ThDP [mitochondrial matrix]
ThDP [cytosol]
Reactome.org reaction link: R-HSA-8875838
======
Reaction input - small molecules:
thiamine(1+) diphosphate(3-)
Reaction output - small molecules:
thiamine(1+) diphosphate(3-)
Reactome.org link: R-HSA-8875838