HHH syndrome is caused by defect in:
Correct Answer: Ornithine permease
Description: 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.
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