Transgenic mice carrying chimeric or mutated type III intermediate filament (IF) genes.
Mice carrying chimeric, truncated or mutated genes encoding intermediate filament (IF) proteins type III do not show any detectable severe pathology. However, upon (over)expression of the transgene in the eye lens 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 cytoskeleton-membrane complex. The degree of expression is paralleled by the extent of the damages.
Bloemendal
H
H
Department of Biochemistry, University of Nijmegen, The Netherlands. H.Bloemendal@bioch.kun.nl
Raats
J M
JM
Pieper
F R
FR
Benedetti
E L
EL
Dunia
I
I
D000818:Animals; D002386:Cataract / Q000235:genetics; D003893:Desmin / Q000235:genetics / Q000502:physiology; D007382:Intermediate Filaments / Q000235:genetics* / Q000502:physiology; D007908:Lens, Crystalline; D051379:Mice; D008822:Mice, Transgenic; D009154:Mutation; D011993:Recombinant Fusion Proteins / Q000235:genetics; D014746:Vimentin / Q000235:genetics / Q000502:physiology
D003893:Desmin; D011993:Recombinant Fusion Proteins; D014746:Vimentin