多囊肾病动物模型的研究进展

多囊肾病(Polycystic kidney disease,PKD)是以肾脏充满多个液性囊泡,细胞增殖异常,间质炎细胞浸润及细胞外基质重塑等病理特点为主的遗传性疾病。主要分为常染色体显性多囊肾病(Autosomal dominant polycystic kidney disease,ADPKD)及常染色体隐性多囊肾病(Autosomal recessive polycystic kidney disease,ARPKD)。ADPKD更为常见,发病率约为1:500-1000,约50%的患者到60岁会发展为终末期肾脏病。ARPKD较少见,发病率约为1:20000-1:40000,患者多在婴幼儿时期死亡。目前,一旦多囊肾发展为终末期肾脏病,除了肾脏移植和透析外没有更有效的治疗方法,因此,早期的诊治对延缓多囊肾进展及防止其发展为终末期肾脏病是至关重要的。多囊肾动物模型的建立在研究多囊肾疾病具体发病机制及新药研发中具有重要意义。本文介绍了PKD疾病动物模型的研究进展,包括经典PKD自发模型、化学诱导模型及基因修饰模型。

Research Advance in Animal Models of Polycystic Kidney Diseases

Polycystic kidney disease is a hereditary disease with a number of fluid accumulation cysts, abnormal cellproliferation, infiltration of interstitial inflammatory cell and reconstruction of extracellular matrix. Autosomal dominant polycystickidney disease (ADPKD) and Autosomal recessive polycystic kidney disease (ARPKD) are two major type of PKD. ADPKD is the mostcommon PKD, its incidence is about 1:500-1:1000 and half of the patients developed end-stage renal disease by the age of 60. However,the incidence of ARPKD is relatively rare, about 1:20000-1:40000, Most patients die in the stage of infancy. Currently, there is no moreeffective treatment method for the PKD patients who developed end-stage renal disease, except kidney transplants and dialysis. Earlydiagnosis and treatment is vital for slowing the progression of the disease and prevent end-stage renal disease. The establishment ofpolycystic kidney animal models is of great significance in the study of the pathogenesis of polycystic kidney disease and its possibletherapeutic drugs. This paper reviews the research progress of animal models of PKD, including the classical spontaneous PKD models,chemical induced models and gene modified models.