Knockout of the intermediate filament protein CP49 destabilises the lens fibre cell cytoskeleton and decreases lens optical quality, but does not induce cataract
| Autor: | Alan R. Prescott, A.M. Hutcheson, Jer R. Kuszak, Shigeo Masaki, Alfred Wegener, Aileen Sandilands, Rebecca K. Zoltoski, Roy A. Quinlan |
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| Rok vydání: | 2003 |
| Předmět: |
Optics and Photonics
BFSP2 Biology Cataract law.invention Mice Cellular and Molecular Neuroscience Optics Intermediate Filament Proteins law Lens Crystalline Animals Scattering Radiation Intermediate Filament Protein Eye Proteins Cytoskeleton Intermediate filament Mice Knockout business.industry Sensory Systems Lens (optics) Disease Models Animal Ophthalmology Fiber cell Intermediate Filament Gene Knockout mouse Biophysics sense organs business |
| Zdroj: | Experimental Eye Research. 76:385-391 |
| ISSN: | 0014-4835 |
| DOI: | 10.1016/s0014-4835(02)00330-5 |
| Popis: | In this report, the phenotype associated with the first targeted knockout of the lens specific intermediate filament gene CP49 is described. Several surprising observations have been made. The first was that no cataract was observed despite the fact that the beaded filaments of the lens fibre cells had been disrupted. Light scatter and the lens optical properties had, however, deteriorated in the CP49 knockout lenses compared to litter mate controls. These changes were accompanied by dramatic changes in plasma membrane organisation of the fibre cells as revealed by detailed morphological examinations and providing the second surprising result. The CP49 knockout mouse is therefore an important model to study the functional link between lens transparency, the cytoskeleton and plasma membrane organisation. |
| Databáze: | OpenAIRE |
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