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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\u2010hydroxy\u20102\u2010nonenal (4HNE), a product of lipid peroxidation, and its increased ubiquitination. 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