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Cell type‐specific regulation of ciliary transition zone assembly in vertebrates
Author(s) -
Wiegering Antonia,
Dildrop Renate,
Kalfhues Lisa,
Spychala André,
Kuschel Stefanie,
Lier Johanna Maria,
Zobel Thomas,
Dahmen Stefanie,
Leu Tristan,
Struchtrup Andreas,
Legendre Flora,
Vesque Christine,
SchneiderMaunoury Sylvie,
Saunier Sophie,
Rüther Ulrich,
Gerhardt Christoph
Publication year - 2018
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.15252/embj.201797791
Subject(s) - biology , cilium , caenorhabditis elegans , microbiology and biotechnology , vertebrate , cell type , tetrahymena , zebrafish , embryonic stem cell , model organism , genetics , gene , cell
Ciliopathies are life‐threatening human diseases caused by defective cilia. They can often be traced back to mutations of genes encoding transition zone (TZ) proteins demonstrating that the understanding of TZ organisation is of paramount importance. The TZ consists of multimeric protein modules that are subject to a stringent assembly hierarchy. Previous reports place Rpgrip1l at the top of the TZ assembly hierarchy in Caenorhabditis elegans . By performing quantitative immunofluorescence studies in RPGRIP1L −/− mouse embryos and human embryonic cells, we recognise a different situation in vertebrates in which Rpgrip1l deficiency affects TZ assembly in a cell type‐specific manner. In cell types in which the loss of Rpgrip1l alone does not affect all modules, additional truncation or removal of vertebrate‐specific Rpgrip1 results in an impairment of all modules. Consequently, Rpgrip1l and Rpgrip1 synergistically ensure the TZ composition in several vertebrate cell types, revealing a higher complexity of TZ assembly in vertebrates than in invertebrates.