Aziz N
Department of Pediatrics, Harvard Medical School, Children's Hospital, Boston, MA 02115, USA.
Bioessays. 1995 Aug;17(8):703-12. doi: 10.1002/bies.950170807.
Polycystic kidney disease (PKD) is one of the most prevalent causes of heritable renal failure. The disease is characterized by the occurrence of numerous fluid-filled cysts within the parenchyma of kidney. The cysts are epithelial in origin and expand in size, leading to crowding of normal kidney tissue. Ultimately, there is gross enlargement of the kidneys with loss of normal functions, and death usually occurs because of complications related to renal failure. Animal models of polycystic kidney disease are proving to be extremely useful for studying the molecular basis of renal cyst formation and for the isolation of genes carrying the mutations. This article describes the various animal models of polycystic kidney disease, spontaneously and experimentally derived, that have recently been identified.
多囊肾病(PKD)是遗传性肾衰竭最常见的病因之一。该病的特征是在肾实质内出现大量充满液体的囊肿。囊肿起源于上皮细胞,且会不断增大,导致正常肾组织受压。最终,肾脏会明显肿大,正常功能丧失,通常因肾衰竭相关并发症而死亡。事实证明,多囊肾病动物模型对于研究肾囊肿形成的分子基础以及分离携带突变的基因极为有用。本文介绍了最近发现的各种自发产生和实验诱导产生的多囊肾病动物模型。