Scavenger receptor B1 post-translational modifications in Rett syndrome
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- 作者：Claudia Sticozzi ; Giuseppe Belmonte ; Aless ; ra Pecorelli ; Franco Cervellati ; Silvia Leoncini ; Cinzia Signorini ; Lucia Ciccoli ; Claudio De Felice ; Joussef Hayek ; Giuseppe Valacchi
- 关键词：Oxidative stress ; 4-Hydroxynonenal ; Ubiquitination ; Protein adducts ; HDL ; Cholesterol
- 刊名：FEBS Letters
- 出版年：2013
- 出版时间：11 July, 2013
- 年：2013
- 卷：587
- 期：14
- 页码：2199-2204
- 全文大小：1308 K
文摘
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.