Cell type‐specific regulation of ciliary transition zone assembly in vertebrates

• Antonia Wiegering ^[1] ; Renate Dildrop ^[1] ; Lisa Kalfhues ^[1] ; André Spychala ^[1] ; Stefanie Kuschel ^[1] ; Johanna Maria Lier ^[1] ; Thomas Zobel ^[1] ; Stefanie Dahmen ^[1] ; Tristan Leu ^[2] ; Andreas Struchtrup ^[1] ; Flora Legendre ^[3] ; Christine Vesque ^[4] ; Sylvie Schneider‐Maunoury ^[4] ; Sophie Saunier ^[3] ; Ulrich Rüther ^[1] ; Christoph Gerhardt ^[1]
  1. [1] Heinrich Heine University Düsseldorf

     Heinrich Heine University Düsseldorf

     Kreisfreie Stadt Düsseldorf, Alemania

     
  2. [2] 1 Institute for Animal Developmental and Molecular Biology Heinrich Heine University Düsseldorf Germany; 8Present address: Institute of Physiology University of Duisburg‐Essen Essen Germany
  3. [3] 3 INSERM, U983 Hôpital Necker‐Enfants Malades Paris France; 4 Sorbonne Paris Cité Faculté de Médecine Université Paris‐Descartes Paris France
  4. [4] 5 Paris‐Seine (IBPS) – Developmental Biology Laboratory Institut de Biologie CNRS, UMR7622 INSERM U1156 Paris France; 6 Sorbonne Universités UPMC Univ Paris 06 Paris France

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• Localización: EMBO journal: European Molecular Biology Organization, ISSN 0261-4189, Vol. 37, Nº. 10, 2018, págs. 5-5
• Idioma: inglés
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  • 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.