Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.

摘要: The mechanisms underlying retinal dystrophy in Usher syndrome type I (USH1) remain unknown because mutant mice lacking any of the USH1 proteins—myosin VIIa, harmonin, cadherin-23, protocadherin-15, sans—do not display degeneration. We found here that, macaque photoreceptor cells, all proteins colocalized at membrane interfaces (i) between inner and outer segments rods (ii) microvillus-like calyceal processes segment basolateral region cones. This pattern, conserved humans frogs, was mediated by formation an protein network, which associated with from early embryonic stages growth onwards. By contrast, mouse photoreceptors lacked had no inner–outer interface. suggest that form adhesion belt around humans, defects this structure cause degeneration patients.