Transgenic mice carrying chimeric or mutated type III intermediate filament (IF) genes |
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Authors: | H Bloemendal J M H Raats F R Pieper E L Benedetti I Dunia |
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Institution: | (1) Department of Biochemistry, University of Nijmegen, P.O. Box 9101, NL-6500 HB Nijmegen (The Netherlands), Fax +31 24 3540525, e-mail: H.Bloemendal@bioch.kun.nl, NE;(2) Département Biologie Supramoléculaire et Cellulaire, Institut Jacques Monod du CNRS, UniversitéParis VII, Paris (France), FR |
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Abstract: | 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.
Received 4 November 1996; accepted 15 November 1996 |
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Keywords: | , Transgenic mice, chimeric IF-genes, cataract, |
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