Unknown,Transcriptomics,Genomics,Proteomics

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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. For analysis of lifelong probucol and CoQ10 supplementation effects in B6.Pdss2(kd/kd) missense mutant mice, total RNA was isolated from the livers of B6 mice and from Pdss2(kd/kd) missense mutant mice fed a normal diet, or a diet supplemented with Probucol or CoQ10. Total RNA was individually hybridized to 20 total Illumina Mouse WG-6 v2.0 arrays, which included 5 biological replicates from each of the 4 groupings: untreated B6.Pdss2(kd/kd) missense mutant mice, probucol-treated B6.Pdss2(kd/kd) mice, CoQ10-treated B6.Pdss2(kd/kd) mice, and untreated B6 wild-type mice. Total RNA

ORGANISM(S): Mus musculus

SUBMITTER: Marni Falk 

PROVIDER: E-GEOD-27954 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications


Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellular mechanisms that cause the widely variable clinical findings. Here, we show that focal segmental glomerulopathy-like kidney disease in Pdss2 mutant animals with primary coenzyme Q (CoQ) deficiency is significantly ameliorated by oral treatment with probucol (1% w/w). Preventative effects in missense mutant mice are similar whether fed probucol from weaning or for 3 weeks prior to typical nephri  ...[more]

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