title: 3-Nitrotyrosine as a biomarker for vascular involvement in Fabry disease.
abstract: Enzyme replacement therapy in Fabry disease was initiated in 2001. In a significant proportion of patients, the apparent removal of stored glycosphingolipid from the endothelial cells does not prevent progression of vascular disease. Shu et al. show a link between accumulation of globotriaosylceramide in the endothelial cells and 3-nitrotyrosine formation, indicating endothelial nitric oxide synthase uncoupling. 3-Nitrotyrosine will be useful to better understand Fabry vasculopathy, and to evaluate additional therapeutic interventions targeting oxidative stress.
authors: Service de Nephrologie, Faculté de Médecine, Hôpital Necker-Enfants Malades, Université Paris-Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: Dominique A
authors: DA
authors: Joly
authors: Service de Nephrologie, Faculté de Médecine, Hôpital Necker-Enfants Malades, Université Paris-Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: Jean-Pierre
authors: JP
authors: Grünfeld
mesh_terms: D000818:Animals; D000795:Fabry Disease / Q000150:complications* / Q000378:metabolism*; D006801:Humans; D008297:Male; D014443:Tyrosine / Q000031:analogs & derivatives* / Q000378:metabolism; D014652:Vascular Diseases / Q000209:etiology* / Q000378:metabolism*
chemical_list: C002744:3-nitrotyrosine; D014443:Tyrosine