Expression data from polycystic kidney disease susceptible and resistant rat strains

To facilitate the search for genetic modifiers that modulate ARPKD disease progression and severity, we sought to generate a congenic rat model that carries the PCK Pkhd1 mutation but is resistant to the development of ARPKD. We transferred the Pkhd1 mutation from the PCK rat onto the genetic background of the FHH (Fawn-Hooded Hypertensive) rat. This newly developed strain, called FHH.Pkhd1, showed significant amelioration of renal disease, and delayed onset of biliary abnormalities. To initiate the exploration for genes and pathways that modulate susceptibility to renal cystogenesis, we investigated transcriptional changes in kidneys from PCK, SD, FHH and FHH.Pkhd1 rats by microarray analysis.为筛选调控常染色体隐性遗传性多囊肾病（ARPKD，autosomal recessive polycystic kidney disease）病程进展与严重程度的遗传修饰因子，本研究旨在构建携带PCK来源Pkhd1基因突变且对ARPKD发病具有抗性的同源导入近交系大鼠模型。我们将PCK大鼠的Pkhd1基因突变导入至FHH（Fawn-Hooded Hypertensive，褐发高血压）大鼠的遗传背景中，成功培育出新型品系FHH.Pkhd1。该品系可显著缓解肾脏病变，并延迟胆道异常的发作时间。为探索调控肾囊肿发生易感性的基因与通路，本研究通过基因芯片分析，对PCK、SD、FHH及FHH.Pkhd1大鼠的肾脏组织进行转录组变化检测。