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 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.
Song
Shuhua
S
Center for Ophthalmic Research/Surgery, Brigham and Women's Hospital, and Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA.
Landsbury
Andrew
A
Dahm
Ralf
R
Liu
Yizhi
Y
Zhang
Qingjiong
Q
Quinlan
Roy A
RA
D000375:Aging / Q000235:genetics; D000818:Animals; D002386:Cataract / Q000209:etiology; D005136:Eye Proteins / Q000235:genetics / Q000502:physiology*; D006801:Humans; D007381:Intermediate Filament Proteins / Q000235:genetics / Q000502:physiology*; D007382:Intermediate Filaments / Q000502:physiology*; D007908:Lens, Crystalline / Q000502:physiology*; D051379:Mice; D009154:Mutation; D014746:Vimentin / Q000502:physiology; D038203:alpha-Crystallin B Chain / Q000502:physiology
D005136:Eye Proteins; D007381:Intermediate Filament Proteins; D014746:Vimentin; D038203:alpha-Crystallin B Chain; C079098:filensin; C084808:phakinin