Truncated forms of Pax-6 disrupt lens morphology in transgenic mice.

摘要: RESULTS. Two lines of PD 1 HD mice and three were generated, all which exhibit posterior nuclear and/or cortical cataracts variable severity. The lenses from transgenic for either Pax-6 truncation are smaller more hydrated than normal. Morphologically, the expressing have swollen lens fibers with attenuated ball-and-socket junctions. In contrast, overexpressing composed cell debris, whereas remaining fiber cells appear generally CONCLUSIONS. presence truncated protein in is sufficient to induce cataract a wild-type genetic background. simplest explanation this phenomenon dominant‐ negative effect; however, number other possible mechanisms presented. (Invest Ophthalmol Vis Sci. 2000;41:464 ‐ 473)