| Literature DB >> 26006105 |
Alessandra Pecorelli1, Giuseppe Belmonte2, Ilaria Meloni3, Franco Cervellati2, Concetta Gardi4, Claudia Sticozzi2, Claudio De Felice5, Cinzia Signorini4, Alessio Cortelazzo6, Silvia Leoncini1, Lucia Ciccoli4, Alessandra Renieri7, Henry Jay Forman8, Joussef Hayek6, Giuseppe Valacchi9.
Abstract
CDKL5 mutation is associated with an atypical Rett syndrome (RTT) variant. Recently, cholesterol homeostasis perturbation and oxidative-mediated loss of the high-density lipoprotein receptor SRB1 in typical RTT have been suggested. Here, we demonstrate an altered lipid serum profile also in CDKL5 patients with decreased levels of SRB1 and impaired activation of the defensive system Nrf2. In addition, CDKL5 fibroblasts showed an increase in 4-hydroxy-2-nonenal- and nitrotyrosine-SRB1 adducts that lead to its ubiquitination and probable degradation. This study highlights a possible common denominator between two different RTT variants (MECP2 and CDKL5) and a possible common future therapeutic target.Entities:
Keywords: 4-Hydroxy-2-nonenal; Free radicals; Inducible nitric oxide synthase; Nitrotyrosine; Nuclear factor erythroid 2-related factor 2; Oxidative stress; Scavenger receptor class B; type 1
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Year: 2015 PMID： 26006105 PMCID： PMC5572621 DOI： 10.1016/j.freeradbiomed.2015.05.010
Source DB: PubMed Journal: Free Radic Biol Med ISSN： 0891-5849 Impact factor: 7.376