USH1 proteins in mouse and macaque photoreceptors. (A) Predicted structures of the USH1 proteins. The positions of the immunizing peptides and recombinant proteins used to engineer the new (in bold) and previously reported antibodies, relative to the different USH1 proteins isoforms, are indicated. (B and C) USH1 proteins in the murine inner ear and retina. (B) In the hair cells from cochleas of P3-P5 mice, cadherin-23, harmonin, and sans punctate immunostainings are detected at the tips of the stereocilia. (C) The organization of the outer neural retina is shown on the left. Myosin VIIa is detected in the inner segment (IS) of photoreceptors in wild-type but not in Myo7a^−/− mice. By contrast, no significant cadherin-23 immunolabeling is detected in the mouse retina. (D and E) USH1 proteins in the macaque retina. Prominent immunostainings for protocadherin-15 and cadherin-23 are observed at the junction between the inner and outer (OS) segments of rod and cone photoreceptors. DAPI nuclear staining (blue) delineates the outer nuclear layer (ONL) and inner nuclear layer (INL). OPL, outer plexiform layer. Bars: (B) 2 µm; (C) 20 µm; (D, left) 10 µm; (D [right] and E) 5 µm.