Article
Rpgrip1l controls ciliary gating by ensuring the proper amount of Cep290 at the vertebrate transition zone.
Molecular biology of the cell - 15 Apr 2021
Wiegering Antonia, Dildrop Renate, Vesque Christine, Khanna Hemant, Schneider-Maunoury Sylvie, Gerhardt Christoph
Abstract excerpt
A range of severe human diseases called ciliopathies is caused by the dysfunction of primary cilia. Primary cilia are cytoplasmic protrusions consisting of the basal body (BB), the axoneme, and the transition zone (TZ). The BB is a modified mother centriole from which the axoneme, the microtubule-based ciliary scaffold, is formed. At the proximal end of the axoneme, the TZ functions as the ciliary gate governing...
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