<prompt> HHH syndrome is caused by defect in:Ornithine permeaseOrnithine transcarbamyolaseArginaseArgininosuccinase lyase  <response>: 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. 