Tags: Disease.
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 the blood a condition called hyperammonemia. Ammonia which is formed when proteins are broken down in the body is toxic if the levels become too high. The nervous system is especially sensitive to the effects of excess ammonia.