Functions of the intermediate filament cytoskeleton in the eye lens

Intermediate filaments (IFs) are a key component of the cytoskeleton in virtually all vertebrate cells, including those of the lens of the eye. IFs help integrate individual cells into their respective tissues. This Review focuses on the lens-specific IF proteins beaded filament structural proteins...

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Bibliographic Details
Published in:The Journal of clinical investigation 2009-07, Vol.119 (7), p.1837-1848
Main Authors: Song, Shuhua, Landsbury, Andrew, Dahm, Ralf, Liu, Yizhi, Zhang, Qingjiong, Quinlan, Roy A
Format: Article
Language:English
Subjects:
Aging - genetics
alpha-Crystallin B Chain - physiology
Animals
Aquaporins
Biomedical research
Cataract - etiology
Cataracts
Cells
Cytoskeleton
Eye
Eye Proteins - genetics
Eye Proteins - physiology
Gene mutations
Health aspects
Humans
Intermediate filament proteins
Intermediate Filament Proteins - genetics
Intermediate Filament Proteins - physiology
Intermediate Filaments - physiology
Lens, Crystalline - physiology
Mice
Mutation
Physiological aspects
Proteins
Retina
Review Series
Sutures
Vertebrates
Vimentin - physiology
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Summary:Intermediate filaments (IFs) are a key component of the cytoskeleton in virtually all vertebrate cells, including those of the lens of the eye. IFs help integrate individual cells into their respective tissues. This Review focuses on the lens-specific IF proteins beaded filament structural proteins 1 and 2 (BFSP1 and BFSP2) and their role in lens physiology and disease. Evidence generated in studies in both mice and humans suggests a critical role for these proteins and their filamentous polymers in establishing the optical properties of the eye lens and in maintaining its transparency. For instance, mutations in both BFSP1 and BFSP2 cause cataract in humans. We also explore the potential role of BFSP1 and BFSP2 in aging processes in the lens.
ISSN:0021-9738
1558-8238
DOI:10.1172/jci38277