Volume 4 Supplement 1

Proceedings of the Second International Cilia in Development and Disease Scientific Conference (2014)

Open Access

C2 domains as protein-protein interaction modules in the ciliary transition zone

  • K Remans1,
  • M Bürger1,
  • I Vetter1 and
  • A Wittinghofer1
Cilia20154(Suppl 1):P65

https://doi.org/10.1186/2046-2530-4-S1-P65

Published: 13 July 2015

RPGRIP1 (RPGR interacting protein 1) is mutated in the severe eye disease Leber´s Congenital Amaurosis (LCA), while the structural homologue RPGRIP1L (RPGRIP1-like) is mutated in many different ciliopathies. Both are large multi-domain proteins predicted to interact with RPGR, the Retinitis Pigmentosa G-Protein Regulator. RPGR is mutated in X-linked Retinitis Pigmentosa and located in photoreceptors and in primary cilia. We solved the crystal structure between the RPGR-interacting domain (RID) of RPGRIP1 and RPGR and demonstrate that RPGRIP1L binds to RPGR in a similar mode. RPGRIP1 binding to RPGR affects the interaction with PDEð, the cargo shuttling factor for prenylated ciliary proteins. The RPGRIP1-RID is a type II C2 domain with a canonical ß-sandwich structure. However, it does not bind Ca2+ and/or phospholipids and thus constitutes a new type of protein-protein interaction module. Judging from the large number of C2 domains present in nearly all ciliary transition zone proteins identified, the structure presented here seems to constitute a cilia-specific module present in multi-protein transition zone complexes.

Authors’ Affiliations

(1)
Max Planck Institute for Molecular Physiology

Copyright

© Remans et al. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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