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 v...

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Bibliographic Details
Published in:Cellular and molecular life sciences : CMLS 1997-01, Vol.53 (1), p.1-12
Main Authors: Bloemendal, H, Raats, J M, Pieper, F R, Benedetti, E L, Dunia, I
Format: Article
Language:English
Subjects:
Acuity
Animals
Cataract - genetics
Cataracts
Cell differentiation
Cytoskeleton
Damage
Desmin - genetics
Desmin - physiology
Differentiation
Eye lens
Gene expression
Genes
Intermediate filaments
Intermediate Filaments - genetics
Intermediate Filaments - physiology
Lens, Crystalline
Lenses
Membranes
Mice
Mice, Transgenic
Mutation
Plasma membranes
Proteins
Recombinant Fusion Proteins - genetics
Rodents
Transgenes
Transgenic animals
Transgenic mice
Vimentin - genetics
Vimentin - physiology
Visual acuity
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Summary: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.
ISSN:1420-682X
1420-9071
DOI:10.1007/PL00000571