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Details on Person UniProt:Q9Y619 SLC25A15
| Class:Id | ReferenceGeneProduct:61004 |
|---|---|
| _chainChangeLog | chain:1-301 added on Fri February 6 2015 |
| _displayName | UniProt:Q9Y619 SLC25A15 |
| _timestamp | 2025-02-21 19:10:17 |
| chain | chain:1-301 |
| checksum | 34436A15B105DC53 |
| comment | FUNCTION Mitochondrial ornithine-citrulline antiporter (Probable) (PubMed:12807890, PubMed:22262851). Catalyzes the exchange between cytosolic ornithine and mitochondrial citrulline plus an H(+), the proton compensates the positive charge of ornithine thus leading to an electroneutral transport. Plays a crucial role in the urea cycle, by connecting the cytosolic and the intramitochondrial reactions of the urea cycle (Probable) (PubMed:12807890, PubMed:22262851). Lysine and arginine are also transported by the antiport mechanism (Probable) (PubMed:12807890). In addition, catalyzes an electroneutral exchange of ornithine or lysine for H(+), a reaction driven by the pH gradient across the inner membrane (By similarity).CATALYTIC ACTIVITY L-citrulline(in) + L-ornithine(out) + H(+)(in) = L-citrulline(out) + L-ornithine(in) + H(+)(out)CATALYTIC ACTIVITY L-ornithine(in) + L-arginine(out) = L-ornithine(out) + L-arginine(in)CATALYTIC ACTIVITY L-ornithine(out) + L-lysine(in) = L-ornithine(in) + L-lysine(out)CATALYTIC ACTIVITY L-lysine(out) + H(+)(in) = L-lysine(in) + H(+)(out)CATALYTIC ACTIVITY L-ornithine(out) + H(+)(in) = L-ornithine(in) + H(+)(out)ACTIVITY REGULATION Inhibited by pyridoxal 5'-phosphate as well as by mercurials (mersalyl, p-chloromercuribenzene sulfonate, and mercuric chloride), N-ethylmaleimide and spermine.BIOPHYSICOCHEMICAL PROPERTIES Highly expressed in liver, pancreas, testis, lung and small intestine. Lower levels are detected in spleen, kidney, brain and heart.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the mitochondrial carrier (TC 2.A.29) family. |
| description | recommendedName: Mitochondrial ornithine transporter 1 alternativeName: Solute carrier family 25 member 15 |
| geneName | SLC25A15 ORC1 ORNT1 SP1855 |
| identifier | Q9Y619 |
| isSequenceChanged | FALSE |
| keyword | Disease variant Membrane Mitochondrion Mitochondrion inner membrane Proteomics identification Reference proteome Repeat Transmembrane Transmembrane helix Transport |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 |
| name | SLC25A15 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8992023] ENSEMBL:ENSG00000102743 SLC25A15 [Homo sapiens] |
| secondaryIdentifier | ORNT1_HUMAN Q5VZD8 Q9HC45 |
| sequenceLength | 301 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:70618] SLC25A15 [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959759] SLC25A15 L283F [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959760] SLC25A15 F188del [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959761] SLC25A15 M37R [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959762] SLC25A15 T272L [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959763] SLC25A15 G190D [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959764] SLC25A15 A15V [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959765] SLC25A15 G113C [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959766] SLC25A15 R179* [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9959768] SLC25A15 T32R [mitochondrial inner membrane] [Homo sapiens] |
| (referenceSequence) | [ReplacedResidue:9959673] L-glutamic acid 180 replaced with L-lysine [ReplacedResidue:9959674] glycine 27 replaced with L-arginine [ReplacedResidue:9959675] L-leucine 71 replaced with L-glutamine [ReplacedResidue:9959676] L-alanine 15 replaced with L-valine [ReplacedResidue:9959677] L-threonine 272 replaced with L-leucine [ReplacedResidue:9959678] L-arginine 275 replaced with L-glutamine [FragmentDeletionModification:9959680] Deletion of residues 188 to 188 [ReplacedResidue:9959681] glycine 190 replaced with L-aspartic acid [ReplacedResidue:9959682] L-methionine 273 replaced with L-lysine [NonsenseMutation:9959683] Nonsense mutation at L-arginine 179 |
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No pathways have been reviewed or authored by UniProt:Q9Y619 SLC25A15 (61004)
