Functions of the intermediate filament cytoskeleton in the eye lens
Shuhua Song, Andrew Landsbury, Ralf Dahm, Yizhi Liu, Qingjiong Zhang, Roy A. Quinlan
Shuhua Song, Andrew Landsbury, Ralf Dahm, Yizhi Liu, Qingjiong Zhang, Roy A. Quinlan
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Functions of the intermediate filament cytoskeleton in the eye lens

Abstract

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.

Authors

Shuhua Song, Andrew Landsbury, Ralf Dahm, Yizhi Liu, Qingjiong Zhang, Roy A. Quinlan

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Figure 5

An example of the Y-shaped cataract caused by the E233del BFSP2 mutation.

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An example of the Y-shaped cataract caused by the E233del BFSP2 mutation...
The cataract is typical of the pedigree recently reported with an E233del BFSP2 mutation (53). Notice the white deposits visible in the lenses of this affected individual taken from a previously published pedigree. The white deposits form a Y shape, which is related to the suture pattern made by the abutting apical and basal ends of the lens fiber cells. The different lighting levels (brighter, top panel; less bright, lower panel) accentuate the anterior (bottom panel) and posterior (top panel) sutures and their associated light scattering deposits. Reproduced with permission from Molecular Vision (53).