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Functions of the intermediate filament cytoskeleton in the eye lens
Shuhua Song, … , Qingjiong Zhang, Roy A. Quinlan
Shuhua Song, … , Qingjiong Zhang, Roy A. Quinlan
Published July 1, 2009
Citation Information: J Clin Invest. 2009;119(7):1837-1848. https://doi.org/10.1172/JCI38277.
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Review Series

Functions of the intermediate filament cytoskeleton in the eye lens

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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 3

Assembly and sequence characteristics of BFSP1/2 filaments and native beaded filaments.

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Assembly and sequence characteristics of BFSP1/2 filaments and native be...
(A) The micrograph shows that in vitro, human BFSP2 and bovine BFSP1 form 10-nm filaments when mixed together at a molar ratio of 2:1. The human BFSP2 was produced recombinantly, while the BFSP1 was purified from bovine lenses. In the absence of α-crystallins, the BFSP1 and BFSP2 proteins form 10-nm filaments, supporting their inclusion in the IF superfamily. Both proteins were prepared as described in ref. 20. (B) This micrograph shows the native filaments present in the lens. Two types are apparent: Smooth, 10-nm filaments (arrowheads) are decorated with α-crystallin particles (small arrows). Beaded filaments (large arrows), which also contain α-crystallin particles, are much more abundant and appear untidy in their association with the supporting filament, to the extent that the filament backbone is almost totally obscured. By comparison, the 10-nm filaments are instantly recognizable despite being decorated with α-crystallin particles. These beaded filaments are the structures first described by Maisel and Perry as being lens specific, and it is from these that filensin and CP49 derive the names BFSP1 and BFSP2, respectively. Scale bars: 500 nm.

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ISSN: 0021-9738 (print), 1558-8238 (online)

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