Molecular physiological analyses of renal failure in pediatric cystic renal diseases

在儿童肾脏疾病中，遗传性肾脏疾病如囊性肾脏的发病率有所增加。为了促进对这些疾病的分析，我们进行了一系列的研究，包括正常小鼠肾脏肾小管的长期体外微灌注。小鼠Henle’s袢皮质厚升肢的生理特性，包括外观和经上皮电压均未受到48小时长时间微灌注制备的影响，表明该新技术适用于多种类型的肾小管快速和慢速适应各种实验操作的研究。PCK大鼠肾Henle’s袢皮质厚升肢对加压素的敏感性与正常SD大鼠肾小管相同。虽然由于2011年3月在日本的灾难，我们失去了大部分研究数据，但我们目前的研究经验强烈表明，长期体外微灌注肾小管将为我们进一步了解肾囊性疾病的生理和病理生理提供重要信息。

Among renal diseases in childhood, the rate of hereditary renal diseases such as cystic kidneys has been increased. To promote the analyses of these diseases, we conducted a series of studies including long-term in vitro microperfusion of renal tubules from normal mouse kidneys. The physiological properties of mouse cortical thick ascending limbs of Henle's loop including appearance and transepithelial voltages were not affected by 48 hours long-microperfused preparation, indicating that this new technique is applicable to many types of investigations for adaptation both rapid and slow of renal tubules to various experimental manipulations.It has also been demonstrated the cortical thick ascending limbs of Henle's loop in PCK rat kidneys are as sensitive to vasopressin as those of tubules from normal SD rats.Although we lost major parts of our research data due to disaster in March 2011 in Japan, our present research experiences strongly suggest that long-term in vitro microperfusion of renal tubules will give us important information on the physiology and pathophysiology of renal cystic diseases further.