Fatty Acid Oxidation is Impaired in An Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney Disease.

EBioMedicine(2016)

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摘要
•Adult female mice have slower kidney disease progression than males in an orthologous Pkd1 model of ADPKD.•Omics and in vitro studies suggest Pkd1 deletion causes cell autonomous metabolic changes and fatty acid oxidation impairment.•Transcriptional sexual dimorphism in mouse kidneys is consistent with lipid metabolism modifying PKD progression in females.•Lower lipid content in diet correlates with slower PKD progression.
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关键词
Polycystic kidney disease,Autosomal Dominant Polycystic Kidney Disease,Pkd1,ADPKD,Mouse model,Systems biology,Metabolism,Fatty acid oxidation,Fatty acid oxidation defect,Lipid metabolism,Diet
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