NPHP proteins: gatekeepers of the ciliary compartment

H Omran - Journal of cell biology, 2010 - rupress.org
H Omran
Journal of cell biology, 2010rupress.org
The cilia and the cytoplasm are separated by a region called the transition zone, where
wedge-shaped structures link the microtubule doublets of the axoneme to the ciliary
membrane, thereby forming a ciliary “gate.” In this issue, Craige et al.(J. Cell Biol. doi:
10.1083/jcb. 201006105) demonstrate in Chlamydomonas reinhardtii that Nphp6/cep290,
which is mutated in nephronophthisis (NPHP), is an integral component of these connectors
and maintains the structural integrity of this gate.
The cilia and the cytoplasm are separated by a region called the transition zone, where wedge-shaped structures link the microtubule doublets of the axoneme to the ciliary membrane, thereby forming a ciliary “gate.” In this issue, Craige et al. (J. Cell Biol. doi:10.1083/jcb.201006105) demonstrate in Chlamydomonas reinhardtii that Nphp6/cep290, which is mutated in nephronophthisis (NPHP), is an integral component of these connectors and maintains the structural integrity of this gate.
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