Figure 4.

Dp71 localizes at the membrane of the lens fiber cells and its absence is associated with a loss of fiber cell organization. Immunolocalization of dystrophins (red) and AQP0 (green) in the anterior pole of the lens from wt (A-D) and KO-Dp71 (E-H) mice. Nuclei were counterstained with DAPI. Dystrophins are localized at the membrane of the secondary fiber together with the AQP0. As expected, total dystrophin staining in KO-Dp71 mice lenses was strongly decreased, confirming that Dp71 is the main product of the DMD gene in the crystalline lens. A comparison of the staining obtained for AQP0 in wt (B) and KO-Dp71 (F) mice confirms a strong disorganization of the ultrastructure of the lens fibers in the absence of Dp71. A weak staining remained in the epithelium of KO-Dp71 mice, suggesting that the other products of the DMD gene are mainly expressed by lens epithelial cells. Higher magnification images in wt crystalline lenses (I-K) clearly reveal a colocolalization of both proteins (K: merged) in the membranes of secondary fibers close to the anterior pole of the lens (e: epithelial cells; f: fiber cells; c: core). Scale bar A: 50 μm; Scale bar I:15 μm.