UniProt ID: Q6PGY3 |
FUNCTION: Mitochondrial electroneutral antiporter that exports citrate from the mitochondria into the cytosol in exchange for malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis-aconitate and to a lesser extend cis- aconitate, maleate and succinate (By similarity). Required for proper neuromuscular junction formation (PubMed:26870663). {ECO:0000250|UniProtKB:P53007, ECO:0000269|PubMed:26870663}. CATALYTIC ACTIVITY: Reaction=(S)-malate(in) + citrate(out) = (S)-malate(out) + citrate(in); Xref=Rhea:RHEA:72483, ChEBI:CHEBI:15589, ChEBI:CHEBI:16947; Evidence={ECO:0000250|UniProtKB:P53007}; CATALYTIC ACTIVITY: Reaction=citrate(out) + D-threo-isocitrate(in) = citrate(in) + D-threo- isocitrate(out); Xref=Rhea:RHEA:72471, ChEBI:CHEBI:15562, ChEBI:CHEBI:16947; Evidence={ECO:0000250|UniProtKB:P53007}; CATALYTIC ACTIVITY: Reaction=citrate(out) + succinate(in) = citrate(in) + succinate(out); Xref=Rhea:RHEA:28835, ChEBI:CHEBI:16947, ChEBI:CHEBI:30031; Evidence={ECO:0000250|UniProtKB:P53007}; CATALYTIC ACTIVITY: Reaction=cis-aconitate(in) + citrate(out) = cis-aconitate(out) + citrate(in); Xref=Rhea:RHEA:72475, ChEBI:CHEBI:16383, ChEBI:CHEBI:16947; Evidence={ECO:0000250|UniProtKB:P53007}; CATALYTIC ACTIVITY: Reaction=citrate(out) + trans-aconitate(in) = citrate(in) + trans- aconitate(out); Xref=Rhea:RHEA:72479, ChEBI:CHEBI:15708, ChEBI:CHEBI:16947; Evidence={ECO:0000250|UniProtKB:P53007}; CATALYTIC ACTIVITY: Reaction=citrate(out) + phosphoenolpyruvate(in) = citrate(in) + phosphoenolpyruvate(out); Xref=Rhea:RHEA:72487, ChEBI:CHEBI:16947, ChEBI:CHEBI:58702; Evidence={ECO:0000250|UniProtKB:P53007}; CATALYTIC ACTIVITY: Reaction=citrate(out) + maleate(in) = citrate(in) + maleate(out); Xref=Rhea:RHEA:72491, ChEBI:CHEBI:16947, ChEBI:CHEBI:30780; Evidence={ECO:0000250|UniProtKB:P53007}; SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q8JZU2}; Multi-pass membrane protein {ECO:0000255}. PTM: Possesses a short cleavable presequence, which, however, is found to be dispensable both for targeting to mitochondria and insertion into the inner membrane. However, the presequence is required to keep SLC25A1 in a soluble state and thus in an import-competent state. Mature SLC25A1 lacking the presequence is prone to aggregation. {ECO:0000250|UniProtKB:P32089}. DISRUPTION PHENOTYPE: Morpholino-injected embryos display altered tail morphology, impaired swimming and defective touch-evoked escape responses. Neuromuscular junction development is abnormal and motor axon terminals show short and erratic outgrowth toward the muscle fiber with no evidence of complete synapse formation. In addition, knockdown embryos often show edema of the hindbrain, heart, yolk sac and tail. {ECO:0000269|PubMed:26870663}. SIMILARITY: Belongs to the mitochondrial carrier (TC 2.A.29) family. {ECO:0000255, ECO:0000255|RuleBase:RU000488}. |
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