Article
Oxidative stress and antioxidant response in fibroblasts from Werner and atypical Werner syndromes.
Aging - 1 Mar 2014
Seco-Cervera Marta, Spis Marta, García-Giménez José Luis, Ibañez-Cabellos José Santiago, Velázquez-Ledesma Ana, Esmorís Isabel, Bañuls Sergio, Pérez-Machado Giselle, Pallardó Federico V
Abstract excerpt
Werner Syndrome (WS, ICD-10 E34.8, ORPHA902) and Atypical Werner Syndrome (AWS, ICD-10 E34.8, ORPHA79474) are very rare inherited syndromes characterized by premature aging. While approximately 90% of WS individuals have any of a range of mutations in theWRN gene, there exists a clinical subgroup in which the mutation occurs in the LMNA/C gene in heterozygosity. Although both syndromes exhibit an age-related...
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