HHH syndrome is caused by defect in:
Hyperammonemia-Hyperornithinemia-Homocitrullinemia (HHH) Syndrome: autosomal recessive disorder Defect is in transpo system of ornithine from cytosol into mitochondria Accumulation of ornithine in the cytosol and a deficiency of ornithine inside mitochondria.
Ornithine permease
Ornithine transcarbamyolase
Arginase
Argininosuccinase lyase
Disorders of Amino acid Metabolism