Entrez 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].
UniProt Summary: Ornithine transport across inner mitochondrial membrane, from the cytoplasm to the matrix.
Pfam DomainsGO Terms
Pfam Domains
Mito carr
GO Terms
mitochondrial L-ornithine transmembrane transport
L-ornithine transmembrane transporter activity
L-ornithine transmembrane transport
ornithine transport
urea cycle
urea metabolic process
nitrogen cycle metabolic process
L-alpha-amino acid transmembrane transport
L-amino acid transport
drug transmembrane transport
amino acid transmembrane transport
mitochondrial transmembrane transport
amino acid transport
carboxylic acid transmembrane transport
organic acid transmembrane transport
drug transport
mitochondrial transport
anion transmembrane transport
carboxylic acid transport
organic acid transport
mitochondrial inner membrane
organic anion transport
amide biosynthetic process
anion transport
cation transmembrane transport
cellular amide metabolic process
cation transport
ion transmembrane transport
response to drug
transmembrane transport
ion transport
organonitrogen compound biosynthetic process
cellular nitrogen compound biosynthetic process
small molecule metabolic process
nitrogen compound transport
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)