TRANSGENIC MICE CARRYING CHIMERIC OR MUTATED TYPE-III INTERMEDIATE FILAMENT (IF) GENES

Mice carrying chimeric, truncated or mutated genes encoding intermedia te filament (IF) proteins type III do not show any detectable severe p athology. However, upon (over)expression of the transgene in the eye l ens all animals develop lens opacification (cataract). At the cellular level the loss of visual acuity is preceded by interference with the terminal differentiation of lens fibre cells, plasma membrane damage, distorted assembly of the IF cytoskeleton and perturbation of the cyto skeleton-membrane complex. The degree of expression is paralleled by t he extent of the damages.