Role of the Polycystins in Cell Migration, Polarity, and Tissue Morphogenesis

Cells

Elisa Agnese Nigro²  Maddalena Castelli²  Alessandra Boletta¹ 

[1] Division of Genetics and Cell Biology, Dibit, IRCCS-San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milano, Italy;

关键词: polycystin;    polycystic kidney disease;    epithelial morphogenesis;    cell migration;    cell polarity;    cilia;    renal cyst;    planar cell polarity;   

DOI  :  10.3390/cells4040687

来源: mdpi

【 摘 要 】

Cystic kidney diseases (CKD) is a class of disorders characterized by ciliary dysfunction and, therefore, belonging to the ciliopathies. The prototype CKD is autosomal dominant polycystic kidney disease (ADPKD), whose mutated genes encode for two membrane-bound proteins, polycystin-1 (PC-1) and polycystin-2 (PC-2), of unknown function. Recent studies on CKD-associated genes identified new mechanisms of morphogenesis that are central for establishment and maintenance of proper renal tubular diameter. During embryonic development in the mouse and lower vertebrates a convergent-extension (CE)-like mechanism based on planar cell polarity (PCP) and cellular intercalation is involved in “sculpting” the tubules into a narrow and elongated shape. Once the appropriate diameter is established, further elongation occurs through oriented cell division (OCD). The polycystins (PCs) regulate some of these essential processes. In this review we summarize recent work on the role of PCs in regulating cell migration, the cytoskeleton, and front-rear polarity. These important properties are essential for proper morphogenesis of the renal tubules and the lymphatic vessels. We highlight here several open questions and controversies. Finally, we try to outline some of the next steps required to study these processes and their relevance in physiological and pathological conditions.

【 授权许可】

CC BY   
© 2015 by the authors; licensee MDPI, Basel, Switzerland.