Falk, Nathalie ORCID: 0000-0001-6722-0292, Kessler, Kristin, Schramm, Sinja-Fee, Boldt, Karsten, Becirovic, Elvir ORCID: 0000-0001-8801-0649, Michalakis, Stylianos ORCID: 0000-0001-5092-9238, Regus-Leidig, Hanna, Noegel, Angelika A., Ueffing, Marius, Thiel, Christian T., Roepman, Ronald ORCID: 0000-0002-5178-8163, Brandstaetter, Johann Helmut and Giessl, Andreas ORCID: 0000-0002-9768-2049 (2018). Functional analyses of Pericentrin and Syne-2 interaction in ciliogenesis. J. Cell Sci., 131 (16). CAMBRIDGE: COMPANY BIOLOGISTS LTD. ISSN 1477-9137

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Abstract

Pericentrin (Pcnt) is a multifunctional scaffold protein and mutations in the human PCNT gene are associated with several diseases, including ciliopathies. Pcnt plays a crucial role in ciliary development in olfactory receptor neurons, but its function in the photoreceptorconnecting cilium is unknown. We downregulated Pcnt in the retina ex vivo and in vivo via a virus-based RNA interference approach to study Pcnt function in photoreceptors. ShRNA-mediated knockdown of Pcnt impaired the development of the connecting cilium and the outer segment of photoreceptors, and caused a nuclear migration defect. In protein interaction screens, we found that the outer nuclear membrane protein Syne-2 (also known as Nesprin-2) is an interaction partner of Pcnt in photoreceptors. Syne-2 is important for positioning murine photoreceptor cell nuclei and for centrosomal migration during early ciliogenesis. CRISPR/Cas9mediated knockout of Syne-2 in cell culture led to an overexpression and mislocalization of Pcnt and to ciliogenesis defects. Our findings suggest that the Pcnt-Syne-2 complex is important for ciliogenesis and outer segment formation during retinal development and plays a role in nuclear migration.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Falk, NathalieUNSPECIFIEDorcid.org/0000-0001-6722-0292UNSPECIFIED
Kessler, KristinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schramm, Sinja-FeeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Boldt, KarstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Becirovic, ElvirUNSPECIFIEDorcid.org/0000-0001-8801-0649UNSPECIFIED
Michalakis, StylianosUNSPECIFIEDorcid.org/0000-0001-5092-9238UNSPECIFIED
Regus-Leidig, HannaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Noegel, Angelika A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ueffing, MariusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thiel, Christian T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Roepman, RonaldUNSPECIFIEDorcid.org/0000-0002-5178-8163UNSPECIFIED
Brandstaetter, Johann HelmutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Giessl, AndreasUNSPECIFIEDorcid.org/0000-0002-9768-2049UNSPECIFIED
URN: urn:nbn:de:hbz:38-177262
DOI: 10.1242/jcs.218487
Journal or Publication Title: J. Cell Sci.
Volume: 131
Number: 16
Date: 2018
Publisher: COMPANY BIOLOGISTS LTD
Place of Publication: CAMBRIDGE
ISSN: 1477-9137
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
CENTROSOME PROTEIN; NUCLEAR MIGRATION; COMPLEXES; ARCHITECTURE; MUTATIONS; NESPRIN-2; ENVELOPE; CILIUM; GOLGI; LOCALIZATIONMultiple languages
Cell BiologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/17726

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