Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria.

@article{Inoue1988BiochemicalAO,
  title={Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria.},
  author={Isao Inoue and Takeyori Saheki and K. Kayanuma and Magali Uono and Masahiro Nakajima and Keizo Takeshita and Ryoko Koike and Testuya Yuasa and Tadashi Miyatake and Koro Sakoda},
  journal={Biochimica et biophysica acta},
  year={1988},
  volume={964 1},
  pages={90-5}
}
Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH disorder) is an inherited metabolic disorder which shows peculiar amino acid changes in the serum and urine. The primary defect is considered to be the transport of ornithine across the mitochondrial membrane, but there is no direct evidence for this so far. We have analyzed ornithine transport activities in the liver mitochondria from three patients with HHH disorder. In coupled liver mitochondria we demonstrated low activities of… CONTINUE READING