Endothelin-1 in the brain of patients with galactosialidosis: its abnormal increase and distribution pattern.

@article{Itoh2000Endothelin1IT,
  title={Endothelin-1 in the brain of patients with galactosialidosis: its abnormal increase and distribution pattern.},
  author={Kouichi Itoh and Kiyomitsu Oyanagi and Hitoshi Takahashi and Toshihiro Sato and Yoshio Hashizume and Michie Shimmoto and Hitoshi Sakuraba},
  journal={Annals of neurology},
  year={2000},
  volume={47 1},
  pages={122-6}
}
Endothelin-1 is a peptidic substrate in vitro of lysosomal protective protein/cathepsin A (PPCA) with serine carboxypeptidase activity. Endothelin-1-specific immunoreactivity has been demonstrated to be markedly increased and distributed abnormally in the neurons and glial cells within autopsied brain regions, including the cerebellum, hippocampal formation, and spinal cord, of patients affected with galactosialidosis, a human PPCA deficiency. The genetic defect of the endothelin-1 degrading… CONTINUE READING
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