Mitochondrial ornithine transporter
| Mitochondrial ornithine transporter 1 | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 301 amino acids | |
| Identifier | ||
| Gene name | SLC25A15 | |
| External IDs | ||
| Transporter classification | ||
| TCDB | 2.A.29 | |
| designation | Mitochondrial carrier | |
| Occurrence | ||
| Parent taxon | multicellular animals | |
| Mitochondrial ornithine transporter 2 | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 301 amino acids | |
| Identifier | ||
| Gene name | SLC25A2 | |
| External IDs | ||
| Transporter classification | ||
| TCDB | 2.A.29.19.1 | |
| designation | Mitochondrial carrier | |
The mitochondrial ornithine transporters (ORNT) are two proteins in the mitochondrial membrane of animals that catalyze the transport of ornithine through the membrane in exchange for citrulline . This reaction is part of the urea cycle . Both proteins contain 301 amino acids and are 88 percent identical. ORNT2 is known to be expressed in the liver , kidneys and pancreas . Mutations in SLC25A15 - gene are known and can be transporter deficiency and hyperammonemia ( HHH syndrome result).
The transport equation is:
- Ornithine (outside) + citrulline (inside) ⇒ ornithine (inside) + citrulline (outside)
So it is an antiport .
Individual evidence
- ↑ Homologues at OMA
- ↑ UniProt Q9Y619 , UniProt Q9BXI2
- ↑ Camacho JA, Rioseco-Camacho N, Andrade D, Porter J, Kong J: Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder . In: Mol. Genet. Metab. . 79, No. 4, August 2003, pp. 257-71. PMID 12948741 .
- ↑ Al-Hassnan ZN, Rashed MS, Al-Dirbashi OY, Patay Z, Rahbeeni Z, Abu-Amero KK: Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with stroke-like imaging presentation: clinical, biochemical and molecular analysis . In: J. Neurol. Sci. . 264, No. 1-2, January 2008, pp. 187-94. doi : 10.1016 / j.jns.2007.08.003 . PMID 17825324 .
- ↑ TCDB : 2.A.29
Web links
Wikibooks: Urea Cycle - Learning and Teaching Materials
