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Primary ciliogenesis requires the distal appendage component Cep123

James E Sillibourne1, Ilse Hurbain, Thierry Grand-Perret

  • 1Institut Curie, Centre de Recherche/UMR144 du Centre Nationale de la Recherche Scientifique , 26 rue d'Ulm, F-75248 Paris Cedex 05 , France.

Biology Open
|June 22, 2013
PubMed

Insights

The distal appendage protein Cep123 is essential for initiating primary cilium assembly by ensuring ciliary vesicle formation. This protein is crucial for early ciliogenesis stages but not for maintaining existing primary cilia.

Area of Science:

  • Cell Biology
  • Molecular Biology
  • Biochemistry

Background:

  • Primary cilia are crucial cellular organelles involved in various signaling pathways.
  • Formation of primary cilia requires precise coordination of mother centriole capping and basal body anchoring via distal appendages.

Purpose of the Study:

  • To investigate the role of the distal appendage protein Cep123 in primary cilia formation.
  • To determine the stage of ciliogenesis where Cep123 functions and its interacting partners.

Main Methods:

  • Analysis of primary cilia formation in the absence of Cep123.
  • Investigation of Cep123 interactions with known centriolar satellite proteins.
  • Mapping of protein interaction domains within Cep123.

Main Results:

  • Cep123 is required for the assembly, but not the maintenance, of primary cilia.
  • Loss of Cep123 leads to a failure in ciliary vesicle formation, indicating a role in early ciliogenesis.
  • Cep123 interacts with PCM-1, Cep290, and OFD1, mediated by its C-terminal domain.

Conclusions:

  • Cep123 is a novel and essential component of the primary ciliogenesis pathway.
  • The findings expand the understanding of distal appendage function in initiating primary cilia assembly.
  • Cep123's interactions highlight its role in coordinating early steps of ciliogenesis.

Keywords:
Centriolar satellitesCentrosomeDistal appendagesPrimary cilium

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