SLC25A12,13 exchange L-Glu and L-Asp
L-Asp + L-Glu + H+ → L-Asp + L-Glu + H+
- Reactome
- R-HSA-372448
- Compartment
- cytosol · mitochondrial inner membrane · mitochondrial matrix
- Rhea
- RHEA:70783
Inputs
- L-AspCHEBI:29991, C4H6NO4, mitochondrial matrix
- L-GluCHEBI:29985, C5H8NO4, cytosol
- H+CHEBI:15378, H, cytosol
Outputs
- L-AspCHEBI:29991, C4H6NO4, cytosol
- L-GluCHEBI:29985, C5H8NO4, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- acidic amino acid transmembrane transporter activity of SLC25A12,13 [mitochondrial inner membrane]
Literature
- Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria EMBO J, 2001
- Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues Biochem J, 2000
- The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein Nat Genet, 1999
- Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency Metab Brain Dis, 2002
- Exogenous aralar/slc25a12 can replace citrin/slc25a13 as malate aspartate shuttle component in liver Mol Genet Metab Rep, 2023
- Metabolic basis and treatment of citrin deficiency J Inherit Metab Dis, 2021