Expression data of cystic renal epithelial tissue from mice deficient for fumarate hydratase

Fumarate hydratase (FH) mutations cause hereditary leiomyomatosis and renal cell cancer (HLRCC). We have conditionally inactivated the murine ortholog (Fh1) in renal tubular epithelial cells in order to generate an in vivo model of HLRCC. Fh1 knockout mice recapitulates important aspects of HLRCC including the development of renal cysts that overexpress hypoxia inducible factor alpha (Hifa) and Hif-target genes. We used microarrays to detail the global programme of gene expression underlying cyst development in Fh1 knockout mice and identified distinct classes of up-regulated genes during this process. Keywords: gene expression, comparison (wild-type n=3 vs knockout n=3) Renal epithelial tissue was macro-dissected from Fh1 knockout mice and sex-matched litter mate control disease-free animals for RNA extraction and hybridization on Affymetrix microarrays.延胡索酸水合酶（Fumarate hydratase, FH）突变可引发遗传性平滑肌瘤病和肾细胞癌（hereditary leiomyomatosis and renal cell cancer, HLRCC）。本研究通过在肾小管上皮细胞中条件性敲除小鼠同源基因（Fh1），以构建HLRCC的体内模型。Fh1敲除小鼠可重现HLRCC的关键病理特征，包括过表达缺氧诱导因子α（hypoxia inducible factor alpha, Hifa）及其靶基因的肾囊肿形成。本研究利用基因芯片（microarrays）技术，解析了Fh1敲除小鼠肾囊肿发生过程中全面的基因表达调控程序，并鉴定出该过程中不同类别的上调基因。关键词：基因表达，对照实验（野生型n=3 vs 敲除型n=3）。研究人员从Fh1敲除小鼠以及性别匹配的同窝无病对照小鼠中大体分离肾上皮组织，用于RNA提取，并在Affymetrix基因芯片上进行杂交实验。