| First Author | Falk N | Year | 2018 |
| Journal | J Cell Sci | Volume | 131 |
| Issue | 16 | PubMed ID | 30054381 |
| Mgi Jnum | J:264301 | Mgi Id | MGI:6195355 |
| Doi | 10.1242/jcs.218487 | Citation | Falk N, et al. (2018) Functional analyses of Pericentrin and Syne-2 interaction in ciliogenesis. J Cell Sci 131(16):jcs218487 |
| abstractText | 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 photoreceptor-connecting 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/Cas9-mediated 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. |
