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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 6

Schematic showing the links between beaded filaments and IFs at different plasma membrane sites in the lens fiber cell.

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Schematic showing the links between beaded filaments and IFs at differen...
Beaded filaments interact with the plasma membranes of lens fiber cells and are often tightly associated, resisting even alkali extraction (111). Two different protein complexes have been characterized recently in lens fiber cells (68), termed the cadherin- and EPPD-based (ezrin, periplakin, periaxin, desmoyokin–based) complexes, which associate with the short and long faces of fiber cells (see Figure 2), respectively. Plakoglobin and plectin are potential linkers for the IFs and beaded filaments to the cadherin complex. On the long faces of the fiber cells, a number of potential proteins that would link the IFs and beaded filaments to the plasma membrane have been identified (FERM, ankyrin), but the identity of the transmembrane proteins has yet to be determined. Although CD44 homologs were looked for, none were found (68), but the lens does express band 3 (96), which could link to ankyrin and spectrin. Some details of this lens plasma membrane–cytoskeleton complex might parallel that of the erythrocyte.

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