CBL228 | Anti-Filamin Antibody, clone PM6/317

CBL228
100 µg  
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      Overview

      Replacement Information

      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, Gp, Ch, Rb, R WB, IHC, IH(P) M Purified Monoclonal Antibody
      Description
      Catalogue NumberCBL228
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Filamin Antibody, clone PM6/317
      Alternate Names
      • Alpha-Filamin
      • Filamin I
      • Endothelial Actin-binding Protein
      • ABP-280
      • Nonmuscle Filamin
      Background InformationFilamin is a structural protein that forms flexible cross-links between two actin filaments. Filamin is a homodimer of polypeptide chains each joined to the other at one end with an actin binding site ath the other. It is present in smooth muscle, fibroblasts, platelets and lymphocytes.
      References
      Product Information
      FormatPurified
      PresentationProtein A affinity purified. The monoclonal is presented at a concentration of 100μg/ml in phosphate buffered saline containing 10mM sodium azide and

      1mg/ml bovine serum albumin.

      We recommend that each laboratory determine an optimum working titre for use in its particular application.
      Applications
      ApplicationThis Anti-Filamin Antibody, clone PM6/317 is validated for use in WB, IH, IH(P) for the detection of Filamin.
      Key Applications
      • Western Blotting
      • Immunohistochemistry
      • Immunohistochemistry (Paraffin)
      Application NotesImmunoblotting: 1:1000-1:4000. Because of the large size of the unprocessed forms of filamin, 4-7% PAGE gels and proteinase inhibitors are recommended.

      Immunoprecipitation: Suggested lysis buffer is PBS with 0.5% triton X-100 with proteinase inhibitors (note for full length filamin include calpain inhibitors). 5 microliters of antibody for every 300-500μL of cell lysate (200-500μg/mL total protein is suggested. Incubation is 1 hour RT or overnight 4C; Protein A/G agarose beads or rabbit anti-mouse secondary capture antibody is recommended for best recovery. 4-8% acrylamide gels are recommended for full length filamin or the 190kDa fragement visualization.

      Immunofluorescence: 1:50-1:200. Suitable for staining both frozen and paraffin embedded tissues (at lower dilutions). Microwave-citrate buffer antigen retrieval method recommended for paraffin sections. Staining is cytoplasmic.

      Optimal working dilutions must be determined by end user.
      Biological Information
      ImmunogenPlatelet filamin
      ClonePM6/317
      HostMouse
      SpecificityRecognizes unprocessed, full length Human filamin (actin-binding protein; 270-280 kDa) as well as the 190 kDa N-terminal calpain cleavage fragment of filamin (Aakhus, 1992). Following induction of apoptosis in U937 cells, the antibody recognizes 170, 150, and 120 kDa N-terminal cleavage fragments of the full-length form presumably resulting from cleavage by activated caspase-3 (Umeda, 2001).
      IsotypeIgG1
      Species Reactivity
      • Human
      • Guinea Pig
      • Chicken
      • Rabbit
      • Rat
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryActin-binding protein, or filamin, is a 280-kD protein that crosslinks actin filaments into orthogonal networks in cortical cytoplasm and participates in the anchoring of membrane proteins for the actin cytoskeleton. Remodeling of the cytoskeleton is central to the modulation of cell shape and migration. Filamin A, encoded by the FLNA gene, is a widely expressed protein that regulates reorganization of the actin cytoskeleton by interacting with integrins, transmembrane receptor complexes, and second messengers.[supplied by OMIM]
      Gene Symbol
      • FLNA
      • Alpha-filamin
      • OPD2
      • MNS
      • FLN
      • FMD
      • NHBP
      • ABP-280
      • Filamin-A
      • OPD1
      • FLN1
      • ABPX
      • OPD
      • Filamin-1
      • DKFZp434P031
      UniProt Number
      UniProt SummaryFUNCTION: SwissProt: P21333 # 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 (By similarity).
      SIZE: 2647 amino acids; 280739 Da
      SUBUNIT: Interacts with PDLIM2 (By similarity). Homodimer. Interacts with FLNB, FURIN, HSPB7, INPPL1, KCND2, MYOT, MYOZ1, ARHGAP24, PSEN1 and PSEN2. Interacts also with various other binding partners in addition to filamentous actin.
      SUBCELLULAR LOCATION: Cytoplasm, cell cortex.
      TISSUE SPECIFICITY: Ubiquitous.
      DOMAIN: SwissProt: P21333 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.
      PTM: Phosphorylated upon DNA damage, probably by ATM or ATR (By similarity). Phosphorylation extent changes in response to cell activation. & The N-terminus is blocked.
      DISEASE: SwissProt: P21333 # Defects in FLNA are the cause of periventricular nodular heterotopia 1 (PVNH1) [MIM:300049]; also called nodular heterotopia, bilateral periventricular (NHBP or BPNH). PVNH1 is an X-linked developmental dominant disorder in which many neurons fail to migrate into the cerebral cortex. They remain as nodules lining the ventricular surface. In heterozygous females these neurons presumably represent those cells that, after X-chromosome inactivation, contain the active X chromosome with the filamin mutation. Most hemizygous affected males die early during embryogenesis, whereas heterozygous females have normal intelligence but suffer from seizures and various manifestations outside the central nervous system, especially related to the vascular system. This implies that essential embryonic cell migration can only occur in FLNA-expressing cells. & Defects in FLNA are the cause of periventricular nodular heterotopia 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 dilatation 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 associated with cerebrofrontofacial syndrome [MIM:608578]. This syndrome consists of a phenotype of male PVNH, with relatively normal development, no epilepsy or other neurological abnormality, severe constipation, and facial dysmorphism and without a discernible skeletal phenotype. & Defects in FLNA are a cause of X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX) [MIM:300048]. CIIPX is caused by severe abnormality of gastrointestinal motility either due to primary qualitative defects of enteric ganglia and nerve fibers, or secondary to a variety of conditions, such as myopathies, inflammatory or autoimmune diseases, drug toxicity, ischemia, or viral infections. CIIPX is diagnosed by radiological, surgical, or manometric evidence of abnormal bowel motility causing intestinal obstruction in the absence of any mechanical occlusion.
      SIMILARITY: Belongs to the filamin family. & Contains 1 actin-binding domain. & Contains 2 CH (calponin-homology) domains. & Contains 24 filamin repeats.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsFor use within 1 month of purchase store at +4°C; for long term

      storage aliquot antibody into small volumes and store at -20°C.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      MOUSE ANTI-HUMAN FILAMIN MONOCLONAL ANTIBODY - 2387530 2387530
      MOUSE ANTI-HUMAN FILAMIN MONOCLONAL ANTIBODY -2608411 2608411
      MOUSE ANTI-HUMAN FILAMIN MONOCLONAL ANTIBODY -2629011 2629011
      MOUSE ANTI-HUMAN FILAMIN MONOCLONAL ANTIBODY -2721679 2721679

      References

      Reference overviewPub Med ID
      Limited proteolysis of filamin is catalyzed by caspase-3 in U937 and Jurkat cells.
      Umeda, T, et al.
      J. Biochem., 130: 535-42 (2001)  2001

      Show Abstract
      11574073 11574073

      Data Sheet

      Title
      Anti-Filamin, clone PM6/317 - Data Sheet

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      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies