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HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes

X-adrenoleukodystrophy (X-ALD) is a peroxisomal metabolic disorder caused by mutations in the ABCD1 gene encoding the peroxisomal ABC transporter adrenoleukodystrophy protein (ALDP). The consistent metabolic abnormality in all forms of X-ALD is an inherited defect in the peroxisomal β-oxidation of v...

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Autores principales: Singh, Jaspreet, Khan, Mushfiquddin, Singh, Inderjit
Formato: Artigo
Lenguaje:Inglês
Publicado: The American Society for Biochemistry and Molecular Biology 2011
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3196237/
https://ncbi.nlm.nih.gov/pubmed/21891797
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.M017491
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