NPHP proteins: gatekeepers of the ciliary compartment.
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.
Omran
Heymut
H
Klinik und Poliklinik für Kinder- und Jugendmedizin, Allgemeine Pädiatrie, Universitätsklinikum Münster 48149, Germany. Heymut.Omran@ukmuenster.de
D000818:Animals; D054468:Axoneme / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D016825:Chlamydomonas reinhardtii / Q000235:genetics / Q000378:metabolism / Q000648:ultrastructure; D002923:Cilia / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D052177:Kidney Diseases, Cystic / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D016253:Microscopy, Immunoelectron; D009154:Mutation; D011506:Proteins / Q000235:genetics* / Q000378:metabolism*; D012162:Retinal Degeneration / Q000378:metabolism / Q000473:pathology
D011506:Proteins
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