The molecular function(s) of nephrocystin-4

点击翻译按钮获取中文摘要

Nephronophthisis (NPH), a hereditary disorder associated with cystic kidney disease and renal failure, has been termed ciliopathy based on the observation that the gene products (nephrocystins) localize to the primary cilium. However, the cellular pathways controlled by nephrocystins are largely unknown. This proposal aims to study the function(s) of nephrocystin-4. Using the developing Xenopus epidermis as a model system, the role of nephrocystin-4 will be analyzed in basal body docking, ciliogenesis, and ciliary polarization. Since nephrocystin-4 interacts with actin-modifying proteins, elucidating the role of nephrocystin-4 in rearrangements of the actin cytoskeleton represents another important part of this study. Addressing the question which cellular programs are controlled by nephrocystin-4, this proposal will examine the role of nephrocystin-4 in cell migration and cell orientation. The analysis of nephrocystin-4 functions will provide further insight into the pathogenesis of NPH, and may yield novel therapeutic targets.