ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)
L-Cit + L-Orn + H+ → L-Cit + L-Orn + H+
- Reactome
- R-HSA-70634
- Compartment
- cytosol · mitochondrial inner membrane · mitochondrial matrix
- Rhea
- RHEA:70787
Inputs
- L-CitCHEBI:57743, C6H13N3O3, mitochondrial matrix
- L-OrnCHEBI:46911, C5H13N2O2, cytosol
- H+CHEBI:15378, H, mitochondrial matrix
Outputs
- L-CitCHEBI:57743, C6H13N3O3, cytosol
- L-OrnCHEBI:46911, C5H13N2O2, mitochondrial matrix
- H+CHEBI:15378, H, cytosol
Catalysed by
- L-ornithine transmembrane transporter activity of Mitochondrial ornithine transporters [mitochondrial inner membrane]
Literature
- Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter. Nat Genet, 1999
- The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms J Biol Chem, 2003
- 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 Mol Genet Metab, 2003