Animal models with cystic kidneys

作者：Neudecker S^*; Gretz N; Hoffmann S

来源：Medizinische Genetik, 2010, 22(3): 332-338.

DOI：10.1007/s11825-010-0230-3

• Polycystic kidney diseases
• Models
• animal
• Cilia
• Cystoproteins
• Ciliopathy

Polycystic kidney disease (PKD) is the most common genetic cause for end-stage renal failure. Numerous fluid-filled cysts develop in the parenchyma of the kidney. They compromise kidney function with increasing number and size of the cysts until renal failure is inevitable. The cysts are epithelial in origin but cysts develop in different nephron segments depending on the type of the PKD. Animal models with PKD have been used for several decades to unravel the molecular mechanisms of cystogenesis. Initially, research was dependent on the morphological analysis of spontaneously emerging cystic phenotypes. Nowadays, in addition to theses models transgenic and knock-out models targeting PKD genes are also available. The localization of "cystoproteins" in the cilia of the tubulus epithelia and the involvement of cilia-dependent pathways in cystogenesis was shown only with the help of these animal models. This article gives an overview on the currently available murine models presenting with PKD.

• 出版日期2010-9
• 单位河北医科大学