Protein Name solute carrier family 25 member 15
Organism Homo sapiens
Gene ID 10166
Gene Symbol

SLC25A15

UniProt Q9Y619 (ORNT1_HUMAN)
Relationships Total Number of functionally related compound(s) : 9
Total Number of Articles : 14
Description

solute carrier family 25 member 15

Gene 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]

synonyms
  • mitochondrial ornithine transporter 1
  • hyperammonemia-hyperornithinemia-homocitrullinuria syndrome
  • ornithine carrier 1
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Properties