Fig. 3.
Usher protein localization in rod photoreceptors. All Usher proteins identified thus far have been shown to localize at two regions in the photoreceptors by immunofluorescence microscopy; the region near the connecting cilium and the synaptic region. Two Usher proteins, myosin VIIA and usherinisoform a localize to the retinal pigment epithelium as well (Bhattacharya et al., 2001; Gibbs et al., 2003). Usherinisoform b, VLGR1, whirlin, and SANS have been shown to localize to the periciliary membrane region by immunogold labeling studies, which is a docking area for vesicular trafficking (Maerker et al., 2008). The EAR domain of VLGR1, which is in the middle of the molecule, has been shown to localize to the space between the periciliary basement membrane and the ciliary plasma membrane (McGee et al., 2006). Based on the presence of Usher proteins near the vesicle loading point at the periciliary membrane it has been proposed that they may play a role in vesicle transport between the inner segments and the outer segments of photoreceptors. BB, basal bodies; CC, connecting cilium; G, Golgi; IS, inner segment; MT, microtubules; N, nucleus; OS, outer segment; RPE, retinal pigment epithelium; S, synaptic region; V, vesicles.