Reactome: A Curated Pathway Database

SLC25A19 transports ThDP from cytosol to mitochondrial matrix

Stable Identifier
R-HSA-8875838
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
Summation

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
Participants
Participant Of
This entity is regulated by:
Title Physical Entity Activity
thiamine pyrophosphate transporter activity of SLC25A19 [mitochondrial inner membrane] SLC25A19 [mitochondrial inner membrane] thiamine pyrophosphate transporter activity (0090422)
Orthologous Events