Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice. - Archive ouverte HAL Access content directly
Journal Articles Journal of Cell Biology Year : 2012

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

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Iman Sahly
  • Function : Author
José-Alain Sahel

Abstract

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 retinal degeneration. We found here that, in macaque photoreceptor cells, all USH1 proteins colocalized at membrane interfaces (i) between the inner and outer segments in rods and (ii) between the microvillus-like calyceal processes and the outer segment basolateral region in rods and cones. This pattern, conserved in humans and frogs, was mediated by the formation of an USH1 protein network, which was associated with the calyceal processes from the early embryonic stages of outer segment growth onwards. By contrast, mouse photoreceptors lacked calyceal processes and had no USH1 proteins at the inner-outer segment interface. We suggest that USH1 proteins form an adhesion belt around the basolateral region of the photoreceptor outer segment in humans, and that defects in this structure cause the retinal degeneration in USH1 patients.
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Dates and versions

inserm-00743698 , version 1 (08-04-2013)

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Iman Sahly, Eric Dufour, Cataldo Schietroma, Vincent Michel, Amel Bahloul, et al.. Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.: Usher 1 retinal pathogenesis. Journal of Cell Biology, 2012, 199 (2), pp.381-99. ⟨10.1083/jcb.201202012⟩. ⟨inserm-00743698⟩
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