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Rer1p maintains ciliary length and signaling by regulating -secretase activity and Foxj1a levels.

J Cell Biol.. 2013-03;  200(6):709 - 720
Jurisch-Yaksi N, Rose AJ, Lu H, Raemaekers T, Munck S, Baatsen P, Baert V, Vermeire W, Scales SJ, Verleyen D, Vandepoel R, Tylzanowski P, Yaksi E, de Ravel T, Yost HJ, Froyen G, Arrington CB, Annaert W. VIB Center for the Biology of Disease, KULeuven, 3000 Leuven, Belgium.
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Abstract

Cilia project from the surface of most vertebrate cells and are important for several physiological and developmental processes. Ciliary defects are linked to a variety of human diseases, named ciliopathies, underscoring the importance of understanding signaling pathways involved in cilia formation and maintenance. In this paper, we identified Rer1p as the first endoplasmic reticulum/cis-Golgi-localized membrane protein involved in ciliogenesis. Rer1p, a protein quality control receptor, was highly expressed in zebrafish ciliated organs and regulated ciliary structure and function. Both in zebrafish and mammalian cells, loss of Rer1p resulted in the shortening of cilium and impairment of its motile or sensory f... More

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