SLC25A15 : solute carrier family 25 member 15
Description
The SLC25A15 (solute carrier family 25 member 15) is a protein-coding gene located on chromosome 13.
The SLC25A15 gene instructs the production of mitochondrial ornithine transporter 1, a key player in the urea cycle, a series of reactions in liver cells. This cycle eliminates excess nitrogen, a byproduct of protein breakdown, by converting it into urea, which is then excreted in urine. The urea cycle prevents the buildup of toxic ammonia, especially harmful to the nervous system. Mitochondrial ornithine transporter 1 is responsible for moving ornithine, a molecule involved in the urea cycle, within mitochondria, the energy-producing compartments of cells. Specifically, it transports ornithine across the inner mitochondrial membrane into the mitochondrial matrix, where it participates in the urea cycle.
The protein encoded by this gene is a mitochondrial ornithine-citrulline antiporter. It catalyzes the exchange between cytosolic ornithine and mitochondrial citrulline plus a proton (H+), ensuring electroneutral transport. The protein is crucial for the urea cycle by connecting its cytosolic and mitochondrial reactions. It also transports lysine and arginine via an antiport mechanism. The protein catalyzes an electroneutral exchange of ornithine or lysine for H+, a reaction driven by the pH gradient across the inner membrane.
SLC25A15 is also known as D13S327, HHH, LNC-HC, ORC1, ORNT1.
Associated Diseases
- Hyperornithinemia-hyperammonemia-homocitrullinemia syndrome
- Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
- Ornithine translocase deficiency
