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More than 99% of the vertebrate ocular lens is comprised of terminally differentiated lens fiber cells. Additionally we are shipping Filensin Kits (8) and Filensin Proteins (6) and many more products for this protein.
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The results suggest that the N-terminal domain of CRYAA (show CRYAA Antibodies) is required during in vitro complex formation with filensin and phakinin (show BFSP2 Antibodies).
A novel mutation (c.1042G>A) at exon 7 of BFSP1, which creates a substitution of an aspartate to an asparagine (p.D348N) was identified to be associated with autosomal dominant congenital cataract in a Chinese family.
The crystallin beta cluster on chromosome 22, GJA3 (show GJA3 Antibodies), and BFSP1 play a major role in maintaining lens transparency.
This is the first report of a mutation in the BFSP1 gene associated with human inherited cataracts.
CP49 (show BFSP2 Antibodies) and filensin have been considered robust markers for mouse lens fiber cell differentiation. The data reported here, however, document both proteins in the mouse lens epithelium, but only after 5 weeks of age, when lens epithelial growth and mitotic activity have slowed.
AQP0 (show MIP Antibodies) modulates GJs in the presence of BF proteins to maintain lens transparency and homeostasis.
disruption of the Hsf4 (show HSF4 Antibodies) gene leads to cataracts via at least three pathways: down-regulation of gamma-crystallin, particularly gamma S-crystallin (show CRYGS Antibodies); decreased lens beaded filament expression; and loss of post-translational modification of alpha A-crystallin (show CRYAA Antibodies)
More than 99% of the vertebrate ocular lens is comprised of terminally differentiated lens fiber cells. Two lens-specific intermediate filament-like proteins, CP49 (also known as phakinin) and the protein product of this gene, filensin, are expressed only after fiber cell differentiation has begun. Both proteins are found in a structurally unique cytoskeletal element that is referred to as the beaded filament (BF). Mutations in this gene are the cause of autosomal recessive cortical juvenile-onset cataract. Multiple transcript variants encoding different isoforms have been found for this gene.
, beaded filament structural protein 1, filensin
, lens fiber cell beaded-filament structural protein CP 95
, cytoskeletal protein, 115 KD
, lens fiber cell beaded-filament structural protein CP 115
, lens intermediate filament-like heavy
, CP97 115-kDa
, lens fiber cell beaded-filament structural protein CP 94
, beaded filament structural protein in lens-CP94