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品种反应性 主要应用 宿主 格式 抗体类型 H WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB9276 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Filamin B Antibody 产品信息 格式 Affinity Purified 控制 - HeLa cells
演示 Affinity purified immunoglobulin. Liquid in 0.02 M PBS, 0.25 M NaCl containing 0.1% sodium azide. 应用 应用 Anti-Filamin B Antibody detects level of Filamin B & has been published & validated for use in WB. 主要应用 - Western Blotting
应用说明 Western blot: 0.1 μg/mL on HeLa cell lysate and LAN-1 human neuroblastoma cell lysate. 0.25-0.5 μg/mL on HuVec cell lysate. Reacts with the ~278 kDa Filamin B protein.
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Synthetic peptide from human filamin B. The immunogen peptide corresponds to a sequence near the hinge 2 region of Filamin B (near C-term) and is not present in the two C-terminally truncated splice variants of Filamin B (Var-2 and Var-3). The peptide shows no significant similarity to other antigens in the human protein database including Filamin A or Filamin C. 宿主 Rabbit 特异性 Filamin B 品种反应性 Human 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_001457.2
基因符号 - DKFZp686O033
- FLNB
- FH1
- FLN3
- LRS1
- AOI
- DKFZp686A1668
- ABP-278
- Filamin-B
- SCT
- Fh1
- FLN1L
- TAP
- TABP
- FLN-B
- Beta-filamin
UniProt编号 - O75369
UniProt汇总 FUNCTION: SwissProt: O75369 # Connects cell membrane constituents to the actin cytoskeleton. May promote orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton. Interaction with FLNA may allow neuroblast migration from the ventricular zone into the cortical plate. Various interactions and localizations of isoforms affect myotube morphology and myogenesis. Isoform 6 accelerates muscle differentiation in vitro.
SIZE: 2602 amino acids; 278195 Da
SUBUNIT: Homodimer. Isoform 1 interacts with FBLP1, FLNA, FLNC, GP1BA, INPPL1, ITGB1A, PSEN1 and PSEN2. Isoform 3 interacts with ITGB1A, ITGB1D, ITGB3 and ITGB6. Interacts with MYOT and MYOZ1. Interacts with HBV capsid protein.
SUBCELLULAR LOCATION: Isoform 1: Cytoplasm, cell cortex. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z-disk. Note=In differentiating myotubes, isoform 1, isoform 2 and isoform 3 are localized diffusely throughout the cytoplasm with regions of enrichment at the longitudinal actin stress fiber. In differentiated tubes, isoform 1 is also detected within the Z- lines. & Isoform 2: Cytoplasm, cytoskeleton. Note=Predominantly localized at actin stress fibers. & Isoform 3: Cytoplasm, cytoskeleton. Note=Predominantly localized at actin stress fibers. & Isoform 6: Cytoplasm, cytoskeleton. Note=Polarized at the periphery of myotubes.
TISSUE SPECIFICITY: Ubiquitous. Isoform 1 and isoform 2 are expressed in placenta, bone marrow, brain, umbilical vein endothelial cells (HUVEC), retina and skeletal muscle. Isoform 1 is predominantly expressed in prostate, uterus, liver, thyroid, stomach, lymph node, small intestine, spleen, skeletal muscle, kidney, placenta, pancreas, heart, lung, platelets, endothelial cells, megakaryocytic and erythroleukemic cell lines. Isoform 2 is predominantly expressed in spinal cord, platelet and Daudi cells. Also expressed in thyroid adenoma, neurofibrillary tangles (NFT), senile plaques in the hippocampus and cerebral cortex in Alzheimer disease (AD). Isoform 3 and isoform 6 are expressed predominantly in lung, heart, skeletal muscle, testis, spleen, thymus and leukocytes. Isoform 4 and isoform 5 are expressed in heart.
DOMAIN: "SwissProt: O75369 Comprised of a NH2-terminal actin-binding domain, 24 internally homologous repeats and two hinge regions. Repeat 24 and the second hinge domain are important for dimer formation. The first hinge region prevents binding to ITGA and ITGB subunits.
DISEASE: "SwissProt: O75369 # Interaction with FLNA may compensate for dysfunctional FLNA homodimer in the periventricular nodular heterotopia (PVNH) disorder. & Defects in FLNB are the cause of atelosteogenesis type 1 (AO1) [MIM:108720]; also known as giant cell chondrodysplasia or spondylohumerofemoral hypoplasia. Atelosteogenesis are lethal short-limb skeletal dysplasias with vertebral abnormalities, disharmonious skeletal maturation, poorly modeled long bones and joint dislocations. & Defects in FLNB are the cause of atelosteogenesis type 3 (AO3) [MIM:108721]. Atelosteogenesis are short-limb lethal skeletal dysplasias with vertebral abnormalities, disharmonious skeletal maturation, poorly modeled long bones and joint dislocations. In AO3 recurrent respiratory insufficiency and/or infections usually result in early death. & Defects in FLNB are the cause of boomerang dysplasia [MIM:112310]. This is a perinatal lethal osteochondrodysplasia characterized by absence or underossification of the limb bones and vertebre. Boomerang dysplasia is distinguished from atelosteogenesis on the basis of a more severe defect in mineralisation, with complete absence of ossification in some limb elements and vertebral segments. & Defects in FLNB are the cause of autosomal dominant Larsen syndrome (LRS1) [MIM:150250]. LRS1 is a genetically heterogeneous disorder characterized by multiple joint dislocations, craniofacial abnormalities and accessory carpal bones. & Defects in FLNB are the cause of spondylocarpotarsal synostosis syndrome [MIM:272460]; also known as spondylocarpotarsal syndrome (SCT) or congenital synspondylism or vertebral fusion with carpal coalition or congenital scoliosis with unilateral unsegmented bar. The disorder is characterized by short stature and vertebral, carpal and tarsal fusions."
SIMILARITY: Belongs to the filamin family. & Contains 1 actin-binding domain. & Contains 2 CH (calponin-homology) domains. & Contains 24 filamin repeats.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at 2-8°C in undiluted aliquots for up to 6 months after date of receipt. 包装信息 数量 100 µg
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