本公司经销BFSP2/Phakinin，晶状体蛋白2抗体，克隆类型为polyclonal，宿主来源是Rabbit，BFSP2/Phakinin晶状体蛋白2抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-11015R
英文名称：Anti-BFSP2/Phakinin
中文名称：晶状体蛋白2抗体
其他名称：49 kDa cytoskeletal protein; Beaded filament protein CP49; Beaded filament structural protein 2; Beaded filament structural protein 2, phakinin; Bfps2, Cytoskeletal protein, 49 kD; BFSP2; BFSP2_HUMAN; CP47; CP49; Lens fiber cell beaded filament protein CP 47; Lens fiber cell beaded filament protein CP 49;Lens intermediate filament-like light; LIFL-L; Phakinin; PHAKOSIN.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 46kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, dog
产品介绍：Phakinin is a membrane-associated ａnd cytoskeletal intermediate filament （IF） protein specific to the eye lens. IFs are cytoskeletal structures that typically contain a head, rod ａnd tail domain. Unlike most IFs, Phakinin completely lacks the C-terminal tail domain thus contributing to the unique structure of the beaded filament that is specific to the lens. Phakinin is required for the assembly of beaded filaments ａnd cytoskeletal networks that are important for the long-term maintenance of optical properties ａnd transparency of the lens. Phakinin copolymerizes with Filensin, another IF protein, to form the 10-nm filamentous structures of the beaded filaments. Phakinin is also capable of self-assembling into filament-like structures that form thicker bundles. Mutations in the gene encoding Phakinin can result in lens cataract.Function : Involved in stabilization of lens fiber cell cytoskeleton.Subunit : Associates with BFSP1. Interacts with LGSN.Subcellular Location : Membrane. Cytoplasm. Cytoplasm > cytoskeleton. Membrane- ａnd cytoskeleton-associated.Tissue Specificity : Lens.DISEASE : Defects in BFSP2 are the cause of cataract autosomal dominant BFSP2-related （ADC-BFSP2）; also known as cataract autosomal dominant multiple types 1. Cataract is an opacification of the crystalline lens of the eye that frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, ａnd may be static or progressive. In general, the more posteriorly located ａnd dense an opacity, the greater the impact on visual function. Cataract autosomal dominant BFSP2-related is characterized by a variable phenotype that may or may not be consistent within a family. The opacities can be nuclear, sutural, stellate cortical, lamellar, cortical, nuclear embryonic, Y-sutural, punctate cortical, congenital or with juvenile- ａnd adult-onset.Similarity : Belongs to the intermediate filament family.