Figure 3: Generation and characterization of heterozygous UBE3D+/− mice.

(a) Schematic representation of the UBE3D gene knockout strategy in mice (we obtained UBE3D+/− heterozygous mice, no UBE3D−/− mice survived). (b) Electron microscopy showed more loose retinal pigment epithelium microvilli and more pigment granules (PG) in 3-month-old UBE3D+/− heterozygous mice than in wild-type mice. Scale bars, 2 μm. (c) ERG demonstrated a lower a-amplitude and b-amplitudein 3-month-old heterozygous UBE3D+/− mice than in age-matched controls. N=6. BI, basal infolding; BM, Bruch's membrane; OS, outer segment of receptor cells; MV, microvilli. The bar in the graph represents the s.e.m. Student’s t-test, **P<0.001.