SLC25A10
Function
Catalyzes the electroneutral exchange or flux of physiologically important metabolites such as dicarboxylates (malonate, malate, succinate), inorganic sulfur-containing anions, and phosphate, across mitochondrial inner membrane (PubMed:29211846). Plays an important role in gluconeogenesis, fatty acid metabolism, urea synthesis, and sulfur metabolism, particularly in liver, by supplying the substrates for the different metabolic processes. Regulates fatty acid release from adipocytes, and contributes to systemic insulin sensitivity (By similarity).
Involvement in disease
Mitochondrial DNA depletion syndrome 19
MTDPS19
An autosomal recessive mitochondrial disorder characterized by progressive and severe epileptic encephalopathy, hypotonia, poor spontaneous movements evolving to spastic quadriparesis and dyskinesias, and respiratory complex I deficiency and mitochondrial DNA depletion in skeletal muscle.
None
The disease may be caused by variants affecting the gene represented in this entry.
Sequence Similarities
Belongs to the mitochondrial carrier (TC 2.A.29) family.
Tissue Specificity
Present in high amounts in liver and kidney, and at lower levels in all the other tissues analyzed.
Cellular localization
- Mitochondrion inner membrane
- Multi-pass membrane protein
Alternative names
DIC, SLC25A10, Mitochondrial dicarboxylate carrier, Solute carrier family 25 member 10