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Beaded filament structural protein 1 (BFSP1)
- Target
- BFSP1
- Molecular classification
- Intermediate filament protein, Cytoskeletal protein, Type VI intermediate filament
01
Overview
Beaded filament structural protein 1 (BFSP1), also known as filensin, is a lens-specific intermediate filament-like cytoskeletal protein essential for the correct formation and maintenance of the beaded filament, a unique cytoskeletal structure in differentiated lens fiber cells. BFSP1, together with phakinin (BFSP2), supports lens fiber architecture and transparency by providing biomechanical stability and proper organization of lens cells. Mutations in BFSP1 are associated with various forms of cataract, emphasizing its critical structural role in ocular lens physiology and transparency; however, it is not a therapeutic target or receptor, but rather a structural protein necessary for lens function[1][2][3][6].
- Other names
- FilensinFilensin C-terminal fragmentFilensin N-terminal fragmentCP115LIFL-HCP94CTRCT33Lens fiber cell beaded-filament structural protein CP115Lens intermediate filament-like heavy, cytoskeletal protein, 115 KD
02
Biological functions
Lens fiber cell structural integrityLens transparencyIntermediate filament organizationCell maturationStructural constituent of eye lens
03
Disease associations
Cataract (congenital cataract, autosomal recessive cortical juvenile-onset cataract, cataract 33)Other (lens-related, non-systemic)
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