Sequence in raw or FASTA format:
SLC25A15 solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15 [Homo sapiens (human)]
|Entrez Gene ID||10166|
|Full Name||solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15|
|Synonyms||D13S327, HHH, ORC1, ORNT1|
|General protein information||
|Organism||Homo sapiens (human)|
|Summary||This gene is a member of the mitochondrial carrier family. The encoded protein transports ornithine across the inner mitochondrial membrane from the cytosol to the mitochondrial matrix. The protein is an essential component of the urea cycle, and functions in ammonium detoxification and biosynthesis of the amino acid arginine. Mutations in this gene result in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. There is a pseudogene of this locus on the Y chromosome.[provided by RefSeq, May 2009].|
Hyperornithinemia-hyperammonemia-homocitrullinemia syndrome, 238970
|REACT_13||Metabolism of amino acids and derivatives|
|Homo sapiens (human)||SLC25A15||NP_055067.1|
|Pan troglodytes (chimpanzee)||SLC25A15||XP_003954299.1|
|Macaca mulatta (Rhesus monkey)||SLC25A15||XP_001088596.1|
|Canis lupus familiaris (dog)||SLC25A15||XP_543118.1|
|Bos taurus (cattle)||SLC25A15||NP_001039791.1|
|Mus musculus (house mouse)||Slc25a15||NP_851842.1|
|Rattus norvegicus (Norway rat)||Slc25a15||NP_001041345.1|
|Gallus gallus (chicken)||SLC25A15||NP_001008442.1|
|Danio rerio (zebrafish)||slc25a15a||NP_001074107.1|
|Danio rerio (zebrafish)||LOC565335||NP_001121816.1|
|Drosophila melanogaster (fruit fly)||CG1628||NP_001259423.1|
|Xenopus (Silurana) tropicalis (western clawed frog)||slc25a15||NP_001090866.1|
|GO:0000066||mitochondrial ornithine transport||TAS|
|GO:0006520||cellular amino acid metabolic process||TAS|
|GO:0034641||cellular nitrogen compound metabolic process||TAS|
|GO:0044281||small molecule metabolic process||TAS|
|GO:0005743||mitochondrial inner membrane||TAS|
|GO:0016021||integral component of membrane||IEA|
|GO:0000064||L-ornithine transmembrane transporter activity||TAS|
What is the normal function of the SLC25A15 gene?
The SLC25A15 gene provides instructions for making a protein called a mitochondrial ornithine transporter. This transporter protein is needed for the urea cycle, a series of reactions that occurs in liver cells. The urea cycle processes excess nitrogen, generated when protein is used by the body, into a compound called urea that is excreted by the kidneys. Excreting the excess nitrogen prevents it from accumulating in the form of ammonia, which is toxic, especially to the nervous system.
The mitochondrial ornithine transporter protein moves a molecule called ornithine within the mitochondria (the energy-producing centers in cells). Specifically, this protein transports ornithine across the inner membrane of mitochondria to the region called the mitochondrial matrix, where it participates in the urea cycle.
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