title: Scavenger receptor B1 post-translational modifications in Rett syndrome.
abstract: The modulation of the HDL receptor scavenger receptor B1 (SRB1) was evaluated in skin fibroblasts isolated from patients with Rett syndrome (RTT), a genetic form of infantile autism. Patients showed an altered plasma lipid profile, while their skin fibroblasts showed a dramatic reduction in SRB1 (immunogold, Western blot and immunohistochemistry). The decreased SRB1 levels were demonstrated to be the consequence of its binding with 4-hydroxy-2-nonenal (4HNE), a product of lipid peroxidation, and its increased ubiquitination. Our findings show for the first time a loss of SRB1 in RTT cells and its relationship with a chronic oxidative stress status.
authors.affiliation: Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Sticozzi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Belmonte
authors.forename: Alessandra
authors.initials: A
authors.lastname: Pecorelli
authors.forename: Franco
authors.initials: F
authors.lastname: Cervellati
authors.forename: Silvia
authors.initials: S
authors.lastname: Leoncini
authors.forename: Cinzia
authors.initials: C
authors.lastname: Signorini
authors.forename: Lucia
authors.initials: L
authors.lastname: Ciccoli
authors.forename: Claudio
authors.initials: C
authors.lastname: De Felice
authors.forename: Joussef
authors.initials: J
authors.lastname: Hayek
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Valacchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000447:Aldehydes / Q000378:metabolism; D016022:Case-Control Studies; D002478:Cells, Cultured; D002648:Child; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D015227:Lipid Peroxidation; D008055:Lipids / Q000097:blood; D018384:Oxidative Stress; D015518:Rett Syndrome / Q000378:metabolism*; D051122:Scavenger Receptors, Class B / Q000378:metabolism*; D054875:Ubiquitination; D055815:Young Adult
chemical_list: D000447:Aldehydes; D008055:Lipids; C495124:SCARB1 protein, human; D051122:Scavenger Receptors, Class B; C027576:4-hydroxy-2-nonenal