Overview

  • Product name

  • Description

    Rabbit polyclonal to Filamin B
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Recombinant fragment corresponding to Human Filamin B aa 611-723.
    Sequence:

    KEPGEYAVHIMCDDEDIKDSPYMAFIHPATGGYNPDLVRAYGPGLEKSGC IVNNLAEFTVDPKDAGKAPLKIFAQDGEGQRIDIQMKNRMDGTYACSYTP VKAIKHTIAVVWG


    Database link: O75369

  • Positive control

    • WB: BEWO, NIH/3T3 and NBT-II cell lysates. IHC-P: Human duodenum, kidney, testis, skin, prostate and pancreas tissue. ICC/IF: U-2 OS cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab224334 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/100 - 1/250. Predicted molecular weight: 278 kDa.
IHC-P 1/500 - 1/1000. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • Function

    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.
  • 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.
  • Involvement in disease

    Note=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 (BOOMD) [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 Larsen syndrome (LRS) [MIM:150250]. An osteochondrodysplasia characterized by large-joint dislocations and characteristic craniofacial abnormalities. The cardinal features of the condition are dislocations of the hip, knee and elbow joints, with equinovarus or equinovalgus foot deformities. Spatula-shaped fingers, most marked in the thumb, are also present. Craniofacial anomalies include hypertelorism, prominence of the forehead, a depressed nasal bridge, and a flattened midface. Cleft palate and short stature are often associated features. Spinal anomalies include scoliosis and cervical kyphosis. Hearing loss is a well-recognized complication.
    Defects in FLNB are the cause of spondylocarpotarsal synostosis syndrome (SCT) [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.
  • Sequence similarities

    Belongs to the filamin family.
    Contains 1 actin-binding domain.
    Contains 2 CH (calponin-homology) domains.
    Contains 24 filamin repeats.
  • Domain

    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.
  • Post-translational
    modifications

    ISGylation prevents ability to interact with the upstream activators of the JNK cascade and inhibits IFNA-induced JNK signaling.
  • Cellular localization

    Cytoplasm > cytoskeleton. Polarized at the periphery of myotubes; Cytoplasm > cytoskeleton. Predominantly localized at actin stress fibers and Cytoplasm > cell cortex. Cytoplasm > cytoskeleton. Cytoplasm > myofibril > sarcomere > Z line. 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.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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
    see all

Images

  • Paraffin-embedded human kidney tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded human pancreas tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded human skeletal muscle tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded human prostate tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded human duodenum tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded human skin tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded human testis tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • Anti-Filamin B antibody (ab224334) at 1/100 dilution + BEWO cell lysate

    Developed using the ECL technique.

    Predicted band size: 278 kDa

  • Paraffin-embedded human duodenum tissue stained for Filamin B using ab224334 at 1/500 dilution in immunohistochemical analysis.

  • All lanes : Anti-Filamin B antibody (ab224334) at 1/100 dilution

    Lane 1 : NIH/3T3 (mouse embryonic fibroblast cell line) cell lysate
    Lane 2 : NBT-II cell lysate

    Developed using the ECL technique.

    Predicted band size: 278 kDa

  • PFA-fixed, Triton X-100 permeabilized U-2 OS (human bone osteosarcoma epithelial cell line) cells stained for Filamin B (green) using ab224334 at 4 µg/ml in ICC/IF.

References

ab224334 has not yet been referenced specifically in any publications.

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