概述

  • 产品名称Anti-Filamin A抗体
    参阅全部 Filamin A 一抗
  • 描述
    兔多克隆抗体to Filamin A
  • 特异性This antibody detects endogenous levels of total Filamin A protein.
  • 经测试应用适用于: IHC-Fr, ICC/IF, WB, ELISA, ICCmore details
  • 种属反应性
    与反应: Mouse, Rat, Human
  • 免疫原

    A synthesized non-phosphopeptide derived from human Filamin A around the phosphorylation site of serine 2152 (A-P-SP-V-A).

  • 阳性对照
    • Extracts of 293 cells.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • 存储溶液pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol, 0.87% Sodium chloride
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab51217 in the following tested applications.

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

应用 Ab评论 说明
IHC-Fr Use at an assay dependent concentration.
ICC/IF Use at an assay dependent concentration.
WB 1/300 - 1/1000. Detects a band of approximately 281 kDa (predicted molecular weight: 281 kDa).
ELISA 1/10000.
ICC 1/1000.

靶标

  • 功能Promotes orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton and serves as a scaffold for a wide range of cytoplasmic signaling proteins. Interaction with FLNA may allow neuroblast migration from the ventricular zone into the cortical plate. Tethers cell surface-localized furin, modulates its rate of internalization and directs its intracellular trafficking.
  • 组织特异性Ubiquitous.
  • 疾病相关Defects in FLNA are the cause of periventricular nodular heterotopia type 1 (PVNH1) [MIM:300049]; also called nodular heterotopia, bilateral periventricular (NHBP or BPNH). PVNH is a developmental disorder characterized by the presence of periventricular nodules of cerebral gray matter, resulting from a failure of neurons to migrate normally from the lateral ventricular proliferative zone, where they are formed, to the cerebral cortex. PVNH1 is an X-linked dominant form. Heterozygous females have normal intelligence but suffer from seizures and various manifestations outside the central nervous system, especially related to the vascular system. Hemizygous affected males die in the prenatal or perinatal period.
    Defects in FLNA are the cause of periventricular nodular heterotopia type 4 (PVNH4) [MIM:300537]; also known as periventricular heterotopia Ehlers-Danlos variant. PVNH4 is characterized by nodular brain heterotopia, joint hypermobility and development of aortic dilation in early adulthood.
    Defects in FLNA are the cause of otopalatodigital syndrome type 1 (OPD1) [MIM:311300]. OPD1 is an X-linked dominant multiple congenital anomalies disease mainly characterized by a generalized skeletal dysplasia, mild mental retardation, hearing loss, cleft palate, and typical facial anomalies. OPD1 belongs to a group of X-linked skeletal dysplasias known as oto-palato-digital syndrome spectrum disorders that also include OPD2, Melnick-Needles syndrome (MNS), and frontometaphyseal dysplasia (FMD). Remodeling of the cytoskeleton is central to the modulation of cell shape and migration. FLNA is a widely expressed protein that regulates re-organization of the actin cytoskeleton by interacting with integrins, transmembrane receptor complexes and second messengers. Males with OPD1 have cleft palate, malformations of the ossicles causing deafness and milder bone and limb defects than those associated with OPD2. Obligate female carriers of mutations causing both OPD1 and OPD2 have variable (often milder) expression of a similar phenotypic spectrum.
    Defects in FLNA are the cause of otopalatodigital syndrome type 2 (OPD2) [MIM:304120]; also known as cranioorodigital syndrome. OPD2 is a congenital bone disorder that is characterized by abnormally modeled, bowed bones, small or absent first digits and, more variably, cleft palate, posterior fossa brain anomalies, omphalocele and cardiac defects.
    Defects in FLNA are the cause of frontometaphyseal dysplasia (FMD) [MIM:305620]. FMD is a congenital bone disease characterized by supraorbital hyperostosis, deafness and digital anomalies.
    Defects in FLNA are the cause of Melnick-Needles syndrome (MNS) [MIM:309350]. MNS is a severe congenital bone disorder characterized by typical facies (exophthalmos, full cheeks, micrognathia and malalignment of teeth), flaring of the metaphyses of long bones, s-like curvature of bones of legs, irregular constrictions in the ribs, and sclerosis of base of skull.
    Defects in FLNA are the cause of X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX) [MIM:300048]. CIIPX is characterized by a severe abnormality of gastrointestinal motility due to primary qualitative defects of enteric ganglia and nerve fibers. Affected individuals manifest recurrent signs of intestinal obstruction in the absence of any mechanical lesion.
    Defects in FLNA are the cause of FG syndrome type 2 (FGS2) [MIM:300321]. FG syndrome (FGS) is an X-linked disorder characterized by mental retardation, relative macrocephaly, hypotonia and constipation.
    Defects in FLNA are the cause of terminal osseous dysplasia (TOD) [MIM:300244]. A rare X-linked dominant male-lethal disease characterized by skeletal dysplasia of the limbs, pigmentary defects of the skin and recurrent digital fibroma during infancy. A significant phenotypic variability is observed in affected females.
    Defects in FLNA are the cause of cardiac valvular dysplasia X-linked (CVDX) [MIM:314400]. A rare X-linked heart disease characterized by mitral and/or aortic valve regurgitation. The histologic features include fragmentation of collagenous bundles within the valve fibrosa and accumulation of proteoglycans, which produces excessive valve tissue leading to billowing of the valve leaflets.
  • 序列相似性Belongs to the filamin family.
    Contains 1 actin-binding domain.
    Contains 2 CH (calponin-homology) domains.
    Contains 24 filamin repeats.
  • 结构域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.
  • 翻译后修饰Phosphorylated upon DNA damage, probably by ATM or ATR (By similarity). Phosphorylation extent changes in response to cell activation.
    The N-terminus is blocked.
  • 细胞定位Cytoplasm > cell cortex. Cytoplasm > cytoskeleton.
  • Information by UniProt
  • 数据库链接
  • 别名
    • ABP 280 antibody
    • ABP-280 antibody
    • Actin-binding protein 280 antibody
    • Alpha filamin antibody
    • Alpha-filamin antibody
    • APBX antibody
    • CSBS antibody
    • CVD1 antibody
    • Endothelial actin binding protein antibody
    • Endothelial actin-binding protein antibody
    • Filamin 1 antibody
    • Filamin A alpha antibody
    • Filamin A antibody
    • Filamin-1 antibody
    • Filamin-A antibody
    • FLN antibody
    • FLN-A antibody
    • FLN1 antibody
    • FLNA antibody
    • FLNA_HUMAN antibody
    • FMD antibody
    • MNS antibody
    • NHBP antibody
    • Non muscle filamin antibody
    • Non-muscle filamin antibody
    • OPD antibody
    • OPD1 antibody
    • OPD2 antibody
    • XLVD antibody
    • XMVD antibody
    see all

Anti-Filamin A antibody 图像

  • All lanes : Anti-Filamin A antibody (ab51217) at 1/300 dilution

    Lane 1 : Extracts of 293 cells treated with EGF (200ng/ml, 5mins).
    Lane 2 : Extracts of 293 cells treated with EGF (200ng/ml, 5mins) and peptide.


    Predicted band size : 281 kDa
    Observed band size : 281 kDa
  • ab51217 staining Filamin A - in the IMCD3 cell line from Mouse Kidney cells by ICC/IF (Immunocytochemistry/immunofluorescence). Cells were fixed with paraformaldehyde, permeabilized with Triton X-100 0.1% in PBS and blocked with milk for 15 minutes at 20°C. Samples were incubated with primary antibody (1/200) for 1 hour at 20°C. A diluted(1/1000) Alexa Fluor®568-conjugated Goat anti-rabbit polyclonal was used as the secondary antibody.

    See Abreview

Anti-Filamin A antibody (ab51217)参考文献

This product has been referenced in:
  • Mizuhashi K  et al. Filamin-interacting proteins, Cfm1 and Cfm2, are essential for the formation of cartilaginous skeletal elements. Hum Mol Genet N/A:N/A (2014). Mouse . Read more (PubMed: 24436304) »
  • Adams M  et al. A meckelin-filamin A interaction mediates ciliogenesis. Hum Mol Genet 21:1272-86 (2012). ICC/IF ; Human . Read more (PubMed: 22121117) »

See all 6 Publications for this product

Product Wall

Application Immunocytochemistry/ Immunofluorescence
Sample Human Cell (Fibroblast)
Permeabilization No
Specification Fibroblast
Blocking step Milk as blocking agent for 15 minute(s) · Concentration: 1% · Temperature: 25°C
Fixative Methanol
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提交于 Jun 05 2015

Application Western blot
Sample Dog Cell lysate - whole cell (Epithelial cells)
Loading amount 20 µg
Specification Epithelial cells
Gel Running Conditions Reduced Denaturing
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 3% · Temperature: 25°C
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提交于 Feb 11 2013

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Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Mouse Tissue sections (E15.5 brain)
Specification E15.5 brain
Fixative Paraformaldehyde
Permeabilization No
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: RT°C
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提交于 Nov 01 2012

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Application Immunocytochemistry/ Immunofluorescence
Sample Mouse Cell (IMCD3)
Specification IMCD3
Fixative Paraformaldehyde
Permeabilization Yes - 0.1% Triton X-100 in PBS for 5 mins
Blocking step Milk as blocking agent for 15 minute(s) · Concentration: 1% · Temperature: 20°C
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提交于 Sep 28 2012

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Application Western blot
Sample Rat Cell lysate - whole cell (Rat Dendritic Cells)
Loading amount 25 µg
Specification Rat Dendritic Cells
Gel Running Conditions Reduced Denaturing (6)
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 22°C
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提交于 Sep 06 2010

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Application Western blot
Sample Human Cell lysate - whole cell (Human Macrophages)
Loading amount 25 µg
Specification Human Macrophages
Gel Running Conditions Reduced Denaturing (6%)
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 20°C
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提交于 Sep 06 2010

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Application Immunocytochemistry
Sample Mouse Cultured Cells (3t3 cells)
Specification 3t3 cells
Fixative Formaldehyde
Permeabilization Yes - triton 1%
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 1% · Temperature: rt°C
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提交于 May 15 2009

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Application Western blot
Sample Human Cell lysate - whole cell (HeLa cells)
Loading amount 30 µg
Specification HeLa cells
Gel Running Conditions Reduced Denaturing (6% SDS-PAGE)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 0.5% · Temperature: R/T°C
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提交于 Nov 13 2008

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"