Renal Cell Regulation and Cancer: Tumor Suppressor Networks and the Primary Cilium

Abstract

Cancer affects a large number of people the world over. Cancer is a class of extremely complex diseases that arise from malfunctions in otherwise vital cellular processes, especially those that govern aspects of cellular functions like proliferation, apoptosis or the cell cycle. These processes are delicately balanced in healthy cells and are tightly regulated by a wide array of interconnected cellular signaling pathways. When these processes or their regulators are dysfunctional, tumorigenesis can occur. The primary cilium is a cellular organelle that can be found projecting from the cell surface of the majority of human cells. This organelle has many functions in cell signaling, including playing a role in the development, pattering and growth of cells and tissues. Dysfunction of the cilium can lead to disease. In addition, a number of proteins important for the function of the cilium are also known to be involved in tumorigenesis. One such protein is the von Hippel-Lindau tumor suppressor protein (pVHL). Mutations in the VHL gene that encodes pVHL lead to ciliogenesis defects and are universally found in kidney cancer tumors. The central topic of this book is an investigation of the function of tumor suppressor proteins that are involved in ciliary biology and the connection between their ciliary role and tumorigenesis. I especially focus on pVHL and its central role in kidney cancer.