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SLC25A15 solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15 [Homo sapiens (human)]


RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_014252 Homo sapiens solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15 (SLC25A15), mRNA. GenEZ ORF Cloning On-demand $449.00 14

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Gene Symbol SLC25A15
Entrez Gene ID 10166
Full Name solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15
Synonyms D13S327, HHH, ORC1, ORNT1
General protein information
Preferred Names
mitochondrial ornithine transporter 1
Names
mitochondrial ornithine transporter 1
ornithine transporter 1
solute carrier family 25 member 15
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

13

13q14

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].
Disorder

MIM: 603861

Hyperornithinemia-hyperammonemia-homocitrullinemia syndrome, 238970

mRNA Protein Product Sequence Price Select
NM_014252, 237649033 NP_055067, 7657585 mitochondrial ornithine transporter 1 ORF Sequence $300.00
Reactome
REACT_13Metabolism of amino acids and derivatives
REACT_847Urea cycle
REACT_111217Metabolism
Homo sapiens (human)SLC25A15NP_055067.1
Pan troglodytes (chimpanzee)SLC25A15XP_003954299.1
Macaca mulatta (Rhesus monkey)SLC25A15XP_001088596.1
Canis lupus familiaris (dog)SLC25A15XP_543118.1
Bos taurus (cattle)SLC25A15NP_001039791.1
Mus musculus (house mouse)Slc25a15NP_851842.1
Rattus norvegicus (Norway rat)Slc25a15NP_001041345.1
Gallus gallus (chicken)SLC25A15NP_001008442.1
Danio rerio (zebrafish)slc25a15aNP_001074107.1
Danio rerio (zebrafish)LOC565335NP_001121816.1
Drosophila melanogaster (fruit fly)CG1628NP_001259423.1
Caenorhabditis elegansT10F2.2NP_498094.1
Xenopus (Silurana) tropicalis (western clawed frog)slc25a15NP_001090866.1
Process
IDNameEvidence
GO:0000050urea cycleTAS
GO:0000066mitochondrial ornithine transportTAS
GO:0006520cellular amino acid metabolic processTAS
GO:0034641cellular nitrogen compound metabolic processTAS
GO:0044281small molecule metabolic processTAS
Component
IDNameEvidence
GO:0005743mitochondrial inner membraneTAS
GO:0016021integral component of membraneIEA
Function
IDNameEvidence
GO:0000064L-ornithine transmembrane transporter activityTAS
GeneCards SLC25A15
UniProt Q9Y619
MIM 603861
HGNC 10985
HPRD 04838

GeneRIFs: Gene References Into Functions What's a GeneRIF?

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.

Interactant

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