HHH syndrome.

Autosomal Recessive.
Mutation in ORNT-1,regulating ornithine permease,of mitochondrial membrane.

Leads to accumulation of ornithine in cytosol Hyperornithinemia. Deficiency in mitochondrial ornithine leads to Hyperammonemia, also the Carbamoly phosphate now Combines with Lysine to form Homocitrulline.