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Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice

ABSTRACT: To discern whether the same transcriptional signature of mitochondrial dysfunction previously reported in B6.Alb/cre,Pdss2loxP/loxP liver-conditional knockout mice (Peng, Falk et al. PLoS Genetics, 2008 [PMID 18437205]) was present regardless of Pdss2 mutation type, as well as to assess whether pharmacologic therapies modulate expression of particular pathways, genome-wide transcriptional profiling was performed in liver from B6.Pdss2(kd/kd) missense mutant mice on standard chow or supplemented long-term with either probucol or CoQ10.

ORGANISM(S): Mus musculus

PROVIDER: GSE27954 | GEO | 2011/06/07

SECONDARY ACCESSION(S): PRJNA137737

REPOSITORIES: GEO

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Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice

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