Ornithine translocase is responsible for transporting
ornithine from the
cytosol into the
mitochondria
A mitochondrion (; ) is an organelle found in the Cell (biology), cells of most Eukaryotes, such as animals, plants and Fungus, fungi. Mitochondria have a double lipid bilayer, membrane structure and use aerobic respiration to generate adenosi ...
in the
urea cycle. It is highly expressed in Liver and Pancreas
Pathology
A disorder is associated with
ornithine translocase deficiency
Ornithine translocase deficiency, also called hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, is a rare autosomal recessive urea cycle disorder affecting the enzyme ornithine translocase, which causes ammonia to accumulate in ...
, and a form of
hyperammonemia.
See also
*
Translocase
External links
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Solute carrier family
Urea cycle
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