3-Nitrotyrosine as a biomarker for vascular involvement in Fabry disease.
Level V
Where this comes from
- Record sourced from PubMed, PMID 24978372.
- Also identified by DOI 10.1038/ki.2014.126.
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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.
Medical subject headings
- Fabry Disease
- Tyrosine
- Vascular Diseases