晶状体蛋白2抗体
规格：1尘驳/1尘濒
英文名： BFSP2
别名： 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;
分子量： 46kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human BFSP2/Ph
交叉反应：Human, Mouse, Rat, Dog,
细胞定位：细胞浆 细胞膜
晶状体蛋白2抗体产物介绍：background: 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晶状体蛋白2抗体: 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 晶状体蛋白2抗体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 -onset. Similarity: Belongs to the intermediate filament family. Database links: Entrez Gene: 8419 Human Omim: 603212 Human SwissProt: Q13515 Human Unigene: 659862 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
晶状体蛋白2抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：神经生物学 细胞粘附分子 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid