Song Shuhua, Landsbury Andrew, Dahm Ralf, Liu Yizhi, Zhang Qingjiong, Quinlan Roy A
Center for Ophthalmic Research/Surgery, Brigham and Women's Hospital, and Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA.
J Clin Invest. 2009 Jul;119(7):1837-48. doi: 10.1172/JCI38277. Epub 2009 Jul 1.
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.
中间丝(IFs)几乎是所有脊椎动物细胞(包括眼晶状体细胞)细胞骨架的关键组成部分。中间丝有助于将单个细胞整合到各自的组织中。本综述重点关注晶状体特异性中间丝蛋白珠状丝结构蛋白1和2(BFSP1和BFSP2)及其在晶状体生理和疾病中的作用。在小鼠和人类研究中产生的证据表明,这些蛋白质及其丝状聚合物在建立眼晶状体的光学特性和维持其透明度方面起着关键作用。例如,BFSP1和BFSP2的突变都会导致人类白内障。我们还探讨了BFSP1和BFSP2在晶状体老化过程中的潜在作用。