Ornithine translocase

  • OMIM: 608 157
  • UniProt: Q9BXI2

Mitochondrial ornithine transporter ( ORNT ) are two proteins in the mitochondrial membrane of animals, which catalyze the transport of ornithine in the exchange with citrulline through the membrane. This reaction is part of the urea cycle. Both proteins contain 301 amino acids and is identical to 88 percent. From ORNT2 you know that it is expressed in liver, kidney and pancreas. Mutations in the SLC25A15 gene are known and can lead to transporter deficiency and hyperammonemia (HHH syndrome).

The transport equation is:

It is an antiport.

Human Genome Organisation Mitochondrial carrier Digital Object Identifier
576066
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