endothelin-1 and 5--deoxy-5--phosphonomethyladenosine-phosphate

endothelin-1 has been researched along with 5--deoxy-5--phosphonomethyladenosine-phosphate* in 1 studies

Other Studies

1 other study(ies) available for endothelin-1 and 5--deoxy-5--phosphonomethyladenosine-phosphate

ArticleYear
Endothelin-1 in the brain of patients with galactosialidosis: its abnormal increase and distribution pattern.
    Annals of neurology, 2000, Volume: 47, Issue:1

    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 activity of PPCA is suggested to cause some of the neurological abnormalities of this disease.

    Topics: Adenosine Diphosphate; Adolescent; Adult; beta-Galactosidase; Brain; Child; Child, Preschool; Endothelin-1; Humans; Immunohistochemistry; Lysosomal Storage Diseases; Male

2000