在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

FLNB Antibody

  • 中文名稱:
    FLNB兔多克隆抗體
  • 貨號:
    CSB-PA008725GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
  • 別名:
    ABP 278 antibody; ABP 280 homolog antibody; ABP-278 antibody; ABP-280 homolog antibody; ABP278 antibody; Actin binding like protein antibody; Actin binding protein 278 antibody; Actin-binding-like protein antibody; AOI antibody; Beta filamin antibody; Beta-filamin antibody; DKFZp686A1668 antibody; DKFZp686O033 antibody; Fh1 antibody; Filamin 1 actin binding protein 280 like antibody; Filamin 3 antibody; Filamin B antibody; Filamin B beta actin binding protein 278 antibody; Filamin homolog 1 antibody; Filamin-3 antibody; Filamin-B antibody; FLN B antibody; FLN-B antibody; FLN1L antibody; FLN3 antibody; FLNB antibody; FLNB_HUMAN antibody; LRS1 antibody; SCT antibody; TABP antibody; TAP antibody; Thyroid autoantigen antibody; Truncated ABP antibody; Truncated actin binding protein antibody; Truncated actin-binding protein antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human FLNB
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB,IF
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    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.
  • 基因功能參考文獻:
    1. In conclusion, the authors identify filamin B as a novel host factor that can interact with core protein to promote hepatitis B virus replication in hepatocytes. PMID: 29594956
    2. We report the case of a male patient with Larsen syndrome found to be mosaic for a novel point mutation in FLNB in whom it was possible to provide evidence-based personalized counseling on transmission risk to future offspring. Using dideoxy sequencing, a low-level FLNB c.698A>G, encoding p.(Tyr233Cys) mutation was detected in buccal mucosa and fibroblast DNA. PMID: 28639312
    3. This is the first identified mutation in the dimerization domain of FLNB. This loss-of-function frameshift mutation in FLNB causes autosomal-recessive SCT with rarely reported rib anomalies. This report demonstrates the involvement of rib anomaly in SCT and its causative mutation in the dimerization domain of FLNB. PMID: 28145000
    4. Our results provide evidence for the involvement of FLNB in the pathogenesis of isolated Congenital talipes equinovarusand have expanded the clinical spectrum of FLNB mutations. PMID: 27395407
    5. splicing variants of FLNB are differentially expressed in giant cell tumor cells and may play a role in the proliferation and differentiation of tumor cells. PMID: 27779699
    6. F-actin clustering through the interaction with the mutant FLNB actin-binding domain may limit the cytoskeletal reorganization, preventing normal skeletal development. PMID: 26491051
    7. FLNb enhances invasion of cancer cells through phosphorylation of MRLC and FAK. PMID: 25925610
    8. Polymorphism at rs11720285, rs11130605 and rs9809315, all of which are located either 5' of the transcription start site or in intron 1 of the FLNB gene has been identified as significantly associated with BMD in Caucasian women. PMID: 24176111
    9. study presents two patients with Atelosteogenesis Type I caused by two novel Filamin B (FLNB) mutations affecting the same FLNB residue: c.542G > A, predicting p.Gly181Asp and c.542G > C, predicting p.Gly181Arg PMID: 23401428
    10. VEGF and PKC promote degradation-independent protein ubiquitination of FLNB to control intracellular trafficking of HDAC7. PMID: 23401860
    11. The structure reveals a new hinge in the linker region between actin binding domain (ABD) and the first filamin repeat that is ideally positioned to orient the ABD for actin binding. PMID: 23036857
    12. skeletal dysplasias -associated mutations in the actin-binding domain of filamin B cause cytoplasmic focal accumulations correlating with disease severity. PMID: 22190451
    13. Multiple single nucleotide polymorphisms and haplotypes in FLNB were significantly associated with bone mineral density, with the strongest association between lumbar spine BMD and rs9828717 (p = 0.005). PMID: 19727905
    14. Maternal genes FLNB, HIC1 and ZNF189 were strongly associated with risk of clefting. PMID: 20634891
    15. findings suggest common variants in FLNB have effects on bone structure in women. Although the location of variants having effects is not entirely consistent, variation at 5' end of the gene may reflect effects on levels of FLNB transcription efficiency PMID: 19453265
    16. Different splice variants of filamin-B affect myogenesis, subcellular distribution, and determine binding to integrin [beta] subunits PMID: 11807098
    17. role in vertebral segmentation, joint formation and endochondral ossification PMID: 14991055
    18. mutations cause four distinct disorders of skeletal development PMID: 15054484
    19. We report the identification of mutations in FLNB in two unrelated individuals with boomerang dysplasia. The resultant substitutions, L171R and S235P, lie within the calponin homology 2 region of the actin binding domain and occur at conserved sites. PMID: 15994868
    20. Novel missense mutations within exon 2 and exon 3 of the FLNB gene are associated with atelosteogenesis I and III. PMID: 16752402
    21. Mutations within the FLNB gene is non-random, with clusters of mutations leading to substitutions in the actin-binding domain and filamin repeats 13-17 being the most common cause of Larsen syndrome. PMID: 16801345
    22. Spondylocarpotarsal synostosis syndrome in a patient with a causal FLNB mutation. PMID: 18257094
    23. identifies filamin B as a molecular linker that mediates ICAM-1-driven transendothelial migration PMID: 18809679
    24. These results establish a novel function of filamin B as a molecular scaffold in the JNK signaling pathway for type I IFN-induced apoptosis. PMID: 18815275
    25. FLNB and SBF2 are associated with human stature. PMID: 19039035
    26. anti-inflammatory activity of Serp-1 is mediated through modification of uPAR-linked beta-integrin and filamin B in monocytes, identifying this interaction as a central regulatory axis for inflammation PMID: 19052145
    27. High-resolution X-ray crystal structures of the human filamin B wild type actin-binding domain, plus W148R and M202V mutants. PMID: 19505475

    顯示更多

    收起更多

  • 相關疾病:
    Atelosteogenesis 1 (AO1); Atelosteogenesis 3 (AO3); Boomerang dysplasia (BOOMD); Larsen syndrome (LRS); Spondylocarpotarsal synostosis syndrome (SCT)
  • 亞細胞定位:
    [Isoform 1]: Cytoplasm, cell cortex. Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, stress fiber. Cytoplasm, myofibril, sarcomere, Z line. 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, stress fiber.; [Isoform 3]: Cytoplasm, cytoskeleton, stress fiber.; [Isoform 6]: Cytoplasm, cytoskeleton. Note=Polarized at the periphery of myotubes.
  • 蛋白家族:
    Filamin family
  • 組織特異性:
    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 i
  • 數據庫鏈接:

    HGNC: 3755

    OMIM: 108720

    KEGG: hsa:2317

    STRING: 9606.ENSP00000420213

    UniGene: Hs.476448



主站蜘蛛池模板: 亚洲最大成人综合网720p| 国产精品鲁鲁鲁| 国产成人免费无庶挡视频| 久久久女人与动物群交毛片| 亚洲国产丝袜精品一区| 中文字幕久久久久人妻| mm1313亚洲国产精品无码试看| 免费人成视频在线播放| 亚洲aⅴ天堂av天堂无码麻豆| 天天做天天爱夜夜爽| 丰满岳乱妇在线观看中字| 亚洲欧美综合国产精品二区| 青草国产精品久久久久久| 大黑人交xxxx18视频| 欧美肥胖老妇bbw| 色先锋资源久久综合5566| 天堂网www天堂资源网| 亚洲丁香婷婷久久一区二区| 中文字幕色av一区二区三区| 激情毛片无码专区| av无码人妻无码男人的天堂| 亚洲熟妇无码八v在线播放| 在线播放午夜理论片| 亚欧免费无码aⅴ在线观看 | 久久人人爽人人爽人人片av东京热| 窝窝影院午夜看片| 人妻人人澡人人添人人爽人人玩 | 忘忧草在线社区www中国中文| 国产在视频线精品视频| 国产精品亚洲精品日韩已满| 日韩做a爰片久久毛片a片| 国产精品福利一区二区| 久激情内射婷内射蜜桃| 精品无人区一区二区三区在线| 欧美孕妇xxxx做受欧美88| 2020久热爱精品视频在线观看| 免费看少妇作爱视频| 亚洲熟妇色xxxxx亚洲| 久久久久久久精品成人执小说一白| 欧美人禽zozo动人物杂交| 亚洲最大的成人网|