Anti-Filamin A抗体[SPM182], prediluted (ab54172)

概述

  • 产品名称Anti-Filamin A抗体[SPM182], prediluted
    参阅全部 Filamin A 一抗
  • 描述
    小鼠单克隆抗体[SPM182] to Filamin A, prediluted
  • 经测试应用适用于: IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Goat, Chicken, Guinea pig
  • 免疫原

    Platelet Filamin A

  • 阳性对照
    • NIH3T3 cells. Skin.

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab54172 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-P
  • 应用说明IHC-P: Ready-to-use for 30 min at RT.
    Staining of formalin-fixed tissues requires boiling tissue sections in 10mM citrate buffer, pH 6.0 for 10 min followed by cooling at RT for 20 min.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 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 [SPM182], prediluted 图像

    • ab54172 (prediluted) staining Filamin A in Human skin by Immunohistochemistry, Formalin-fixed, Paraffin embedded tissue.

    Anti-Filamin A antibody [SPM182], prediluted (ab54172)参考文献

    ab54172 has not yet been referenced specifically in any publications.

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