Scavenger receptor B1 post-translational modifications in Rett syndrome
•Rett patients have an alterated plasma lipid profile.•SRB1 (HDL receptor) is low in Rett fibroblasts.•Rett fibroblasts have high level of HNE with formation of HNE–SRB1 adducts.•SRB1 is ubiquitinated. The modulation of the HDL receptor scavenger receptor B1 (SRB1) was evaluated in skin fibroblasts...
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| Published in | FEBS letters Vol. 587; no. 14; pp. 2199 - 2204 |
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| Main Authors | , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
England
Elsevier B.V
11.07.2013
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| Subjects | |
| Online Access | Get full text |
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| Abstract | •Rett patients have an alterated plasma lipid profile.•SRB1 (HDL receptor) is low in Rett fibroblasts.•Rett fibroblasts have high level of HNE with formation of HNE–SRB1 adducts.•SRB1 is ubiquitinated.
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. |
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| AbstractList | 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. •Rett patients have an alterated plasma lipid profile.•SRB1 (HDL receptor) is low in Rett fibroblasts.•Rett fibroblasts have high level of HNE with formation of HNE–SRB1 adducts.•SRB1 is ubiquitinated. 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. 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. Rett patients have an alterated plasma lipid profile. SRB1 (HDL receptor) is low in Rett fibroblasts. Rett fibroblasts have high level of HNE with formation of HNE–SRB1 adducts. SRB1 is ubiquitinated. 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.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. |
| Author | Sticozzi, Claudia Cervellati, Franco Hayek, Joussef Belmonte, Giuseppe Ciccoli, Lucia Valacchi, Giuseppe Leoncini, Silvia Signorini, Cinzia De Felice, Claudio Pecorelli, Alessandra |
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| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/23711372$$D View this record in MEDLINE/PubMed |
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| Snippet | •Rett patients have an alterated plasma lipid profile.•SRB1 (HDL receptor) is low in Rett fibroblasts.•Rett fibroblasts have high level of HNE with formation... The modulation of the HDL receptor scavenger receptor B1 (SRB1) was evaluated in skin fibroblasts isolated from patients with Rett syndrome (RTT), a genetic... |
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| SubjectTerms | 4-Hydroxynonenal Adolescent Adult Aldehydes - metabolism autism blood lipids Case-Control Studies Cells, Cultured Child Cholesterol Female fibroblasts Fibroblasts - metabolism HDL high density lipoprotein Humans immunohistochemistry Lipid Peroxidation Lipids - blood Oxidative Stress patients Protein adducts Rett Syndrome - metabolism Scavenger Receptors, Class B - metabolism Ubiquitination Western blotting Young Adult |
| Title | Scavenger receptor B1 post-translational modifications in Rett syndrome |
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