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。 Polycystic kidney disease (PKD) is a hereditary disease mainly characterized by multiple kidney vacuoles, abnormal cell proliferation, interstitial inflammatory cell infiltration and extracellular matrix remodeling. Mainly divided into autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD is more common, with an incidence of approximately 1: 500-1000. About 50% of patients develop terminal disease of kidney disease by age 60. ARPKD rare, the incidence of about 1: 20000-1: 40000, many patients died in infancy. Currently, once polycystic kidney disease develops into end-stage renal disease, there is no more effective treatment than kidney transplantation and dialysis, so early diagnosis and treatment is crucial for delaying the progression of polycystic kidney disease and preventing its development into end-stage renal disease of. The establishment of polycystic kidney animal model in the study of specific pathogenesis of polycystic kidney disease and the development of new drugs is of great significance. This article describes the progress of animal models of PKD disease, including classical PKD spontaneous model, chemical induced model and gene modification model.