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Shuhua Song, Andrew Landsbury, Ralf Dahm, Yizhi Liu, Qingjiong Zhang, Roy A. Quinlan
J Clin Invest. 2009;
119(7):1837
doi:10.1172/JCI38277
Abstract |
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I
ntermediate 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.
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Citations to this article
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(12)
| Title and authors |
Publication |
Year |
sHSP in the eye lens: Crystallin mutations, cataract and proteostasis
Alice R. Clark, Nicolette H. Lubsen, Christine Slingsby
|
The International Journal of Biochemistry & Cell Biology
|
2012 |
Truncation, cross-linking and interaction of crystallins and intermediate filament proteins in the aging human lens
Shih-Ping Su, Jason D. McArthur, Roger J.W. Truscott, J. Andrew Aquilina
|
Biochimica et Biophysica Acta (BBA) - Proteins & Proteomics
|
2011 |
A New Focus on RNA in the Lens
M. K. Duncan
|
Science
|
2011 |
PutativeArabidopsishomologues of metazoan coiled-coil cytoskeletal proteins
John Gardiner, Robyn Overall, Jan Marc
|
Cell. Biol. Int.
|
2011 |
Periaxin is required for hexagonal geometry and membrane organization of mature lens fibers
Rupalatha Maddala, Nikolai P. Skiba, Robert Lalane, Diane L. Sherman, Peter J. Brophy, Ponugoti V. Rao
|
Developmental Biology
|
2011 |
Novel recessive BFSP2 and PITX3 mutations: Insights into mutational mechanisms from consanguineous populations :
Mohammed A. Aldahmesh, Arif O. Khan, Jawahir Mohamed, Fowzan S. Alkuraya
|
Genetics in Medicine
|
2011 |
Evolution of the vertebrate beaded filament protein, Bfsp2; comparing the in vitro assembly properties of a “tailed” zebrafish Bfsp2 to its “tailless” human orthologue
Qu Bo, Andrew Landsbury, Helia Berrit Schönthaler, Ralf Dahm, Yizhi Liu, John I. Clark, Alan Prescott, Roy A. Quinlan
|
Experimental Eye Research
|
2011 |
Hereditary pediatric cataract on the Arabian Peninsula
Arif O. Khan
|
Saudi Journal of Ophthalmology
|
2011 |
Presbyopia and cataract: A question of heat and time
Roger J.W. Truscott, Xiangjia Zhu
|
Progress in Retinal and Eye Research
|
2010 |
Intermediate filaments take the heat as stress proteins
D.M. Toivola, P. Strnad, A. Habtezion, M.B. Omary
|
Trends in Cell Biology
|
2010 |
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