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MAB2160 | Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3

MAB2160
100 µL  
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      Replacement Information

      Ofertas especiais

      Tabela com principais espec.

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, M, R ELISA, ICC, IHC, IH(P), WB M Ascites Monoclonal Antibody
      Description
      Catalogue NumberMAB2160
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Fragile X Mental Retardation Protein Antibody, clone 1C3
      Alternate Names
      • FMRP
      Background InformationFragile X Mental Retardation Protein (FMRP) is a RNA-binding protein that is associated to polysomes and may be involved in the transport of mRNA from the nucleus to the cytoplasm. Defects in FMR1 are the cause of Fragile X syndrome, which is a common genetic disease characterized by moderate to severe mental retardation, macroorchidism, large ears, prominent jaw, and high-pitched, jocular speech. The defect in most fragile X syndrome patients results from an amplification of a CGG repeat region which is directly in front of the coding region.
      References
      Product Information
      FormatAscites
      Control
      • HeLa whole cell lysate, HeLa nuclear lysate, mouse E17 spinal cord lysate
      PresentationAscites mouse monoclonal IgG1k fluid containing no preservatives
      Applications
      ApplicationAnti-Fragile X Mental Retardation Protein Antibody, clone 1C3 detects level of Fragile X Mental Retardation Protein & has been published & validated for use in ELISA, IC, IH, IH(P) & WB.
      Key Applications
      • ELISA
      • Immunocytochemistry
      • Immunohistochemistry
      • Immunohistochemistry (Paraffin)
      • Western Blotting
      Applications Not Recommended
      • Flow Cytometry
      • Immunoprecipitation
      Application NotesImmunohistochemistry:
      Frozen and paraffin sections. 1:500-1:5,000 dilution of a previous lot was used.

      ELISA:
      A 1:500-1:5,000 dilution of a previous lot was used in ELISA.

      Detection of FMRP on Blood Smears:
      A 1:500-1:5,000 dilution of a previous lot was used.

      Immunocytochemistry:
      A 1:500-1:5,000 dilution of a previous lot was used on transfected cells. Light fixation (2% PFA, permeabilize with 0.1% triton in block only)

      Optimal working dilutions must be determined by the end user.
      Biological Information
      ImmunogenFusion protein with a full length FMRP (human).
      Clone1C3
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityHuman and mouse FMRP. The epitope is localized in the N-terminal half of FMRP. Cross reaction with FXR protein may be detected in cases of high expression of the latter proteins.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      • Mouse
      • Rat
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThe protein encoded by this gene binds RNA and is associated with polysomes. The encoded protein may be involved in mRNA trafficking from the nucleus to the cytoplasm. A trinucleotide repeat (CGG) in the 5' UTR is normally found at 6-53 copies, but an expansion to 55-230 repeats is the cause of fragile X syndrome.
      Gene Symbol
      • FMR1
      • FRAXA
      • FMRP
      • MGC87458
      Purification MethodUnpurified
      UniProt Number
      UniProt SummaryFUNCTION: SwissProt: Q06787 # RNA-binding protein. Associated with polysomes and might be involved in the transport of mRNA from the nucleus to the cytoplasm. Binds strongly to poly(G), binds moderately to poly(U) but shows very little binding to poly(A) or poly(C).
      SIZE: 632 amino acids; 71174 Da
      SUBUNIT: Found in a RNP granule complex with IGF2BP1. Interacts with FXR1, FXR2, IGF2BP1, NUFIP1 and RANBP9. Interacts with CYFIP1 and CYFIP2.
      SUBCELLULAR LOCATION: Cytoplasm. Nucleus.
      TISSUE SPECIFICITY: Highest levels found in neurons, brain, testis, placenta and lymphocytes. Also expressed in epithelial tissues and at very low levels in glial cells.
      DISEASE: SwissProt: Q06787 # Defects in FMR1 are the cause of fragile X syndrome. [MIM:300624]. It is a common genetic disease (has a prevalence of one in every 2000 children) which is characterized by moderate to severe mental retardation, macroorchidism (enlargement of the testicles), large ears, prominent jaw, and high-pitched, jocular speech. The defect in most fragile X syndrome patients results from an amplification of a CGG repeat region which is directly in front of the coding region. & Defects in FMR1 are the cause of fragile X tremor/ataxia syndrome (FXTAS) [MIM:300623]. In FXTAS, the expanded repeats range in size from 55 to 200 repeats and are referred to as 'premutations'. Full repeat expansions with greater than 200 repeats results in fragile X mental retardation syndrome [MIM:300624]. Carriers of the premutation typically do not show the full fragile X syndrome phenotype, but comprise a subgroup that may have some physical features of fragile X syndrome or mild cognitive and emotional problems.
      SIMILARITY: SwissProt: Q06787 ## Belongs to the FMR1 family. & Contains 2 KH domains.
      MISCELLANEOUS: RNA-binding activity is inhibited by RANBP9. & The mechanism of the severe phenotype in the Asn- 304 patient lies in the sequestration of bound mRNAs in nontranslatable mRNP particles. In the absence of FMRP, these same mRNAs may be partially translated via alternate mRNPs, although perhaps abnormally localized or regulated, resulting in typical fragile X syndrome. Asn-304 mutation maps to a position within the second KH domain of FMRP that is critical for stabilizing sequence-specific RNA-protein interactions. Asn-304 mutation abrogates the association of the FMRP KH 2 domain with its target, kissing complex RNA.
      Molecular Weight~71 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot on Mouse E17 spinal cord lysates.

      Western Blotting Analysis:
      1:500 dilution of this antibody detected Fragile X Mental Retardation Protein on 10 μg of Mouse E17 spinal cord lysates.
      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 ConditionsStable for 1 year at -20ºC from date of receipt.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      MSDS

      Título

      Ficha de Segurança de Produtos (MSDS) 

      Certificados de análise

      TítuloNúmero do lote
      Anti-Fragile X Mental Retardation -2548166 2548166
      Anti-Fragile X Mental Retardation -2795019 2795019
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2137991 2137991
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2148229 2148229
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2387453 2387453
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2453146 2453146
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 1943253 1943253
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2000381 2000381
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2008989 2008989
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2055438 2055438
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2272115 2272115
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - 2500405 2500405
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - JC1634185 JC1634185
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - JC1658255 JC1658255
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - JC1668383 JC1668383
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - LV1555755 LV1555755
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - NG1726916 NG1726916
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - NG1749702 NG1749702
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - NG1780761 NG1780761
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - NG1916121 NG1916121
      Anti-Fragile X Mental Retardation Protein, clone 1C3 -2571238 2571238
      Anti-Fragile X Mental Retardation Protein, clone 1C3 -2677892 2677892
      Anti-Fragile X Mental Retardation Protein, clone 1C3 -2709164 2709164
      Anti-Fragile X Mental Retardation Protein, clone 1C3 -2762372 2762372

      Referências | 24 Disponível | Ver todas as referências

      Visão geral das referênciasAplicaçãoPub Med ID
      Conserved hippocampal cellular pathophysiology but distinct behavioural deficits in a new rat model of FXS.
      Till, SM; Asiminas, A; Jackson, AD; Katsanevaki, D; Barnes, SA; Osterweil, EK; Bear, MF; Chattarji, S; Wood, ER; Wyllie, DJ; Kind, PC
      Human molecular genetics 24 5977-84 2015

      Mostrar Resumo
      26243794 26243794
      A novel fragile X syndrome mutation reveals a conserved role for the carboxy-terminus in FMRP localization and function.
      Okray, Z; de Esch, CE; Van Esch, H; Devriendt, K; Claeys, A; Yan, J; Verbeeck, J; Froyen, G; Willemsen, R; de Vrij, FM; Hassan, BA
      EMBO molecular medicine 7 423-37 2015

      Mostrar Resumo
      25693964 25693964
      CNS expression of murine fragile X protein (FMRP) as a function of CGG-repeat size.
      Ludwig, AL; Espinal, GM; Pretto, DI; Jamal, AL; Arque, G; Tassone, F; Berman, RF; Hagerman, PJ
      Human molecular genetics 23 3228-38 2014

      Mostrar Resumo
      24463622 24463622
      FMRP S499 is phosphorylated independent of mTORC1-S6K1 activity.
      Bartley, CM; O'Keefe, RA; Bordey, A
      PloS one 9 e96956 2014

      Mostrar Resumo
      24806451 24806451
      Fragile X mental retardation protein controls synaptic vesicle exocytosis by modulating N-type calcium channel density.
      Ferron, L; Nieto-Rostro, M; Cassidy, JS; Dolphin, AC
      Nature communications 5 3628 2014

      Mostrar Resumo
      24709664 24709664
      A quantitative homogeneous assay for fragile X mental retardation 1 protein.
      Schutzius, G; Bleckmann, D; Kapps-Fouthier, S; di Giorgio, F; Gerhartz, B; Weiss, A
      Journal of neurodevelopmental disorders 5 8 2013

      Mostrar Resumo
      23548045 23548045
      Top3β is an RNA topoisomerase that works with fragile X syndrome protein to promote synapse formation.
      Xu, D; Shen, W; Guo, R; Xue, Y; Peng, W; Sima, J; Yang, J; Sharov, A; Srikantan, S; Yang, J; Fox, D; Qian, Y; Martindale, JL; Piao, Y; Machamer, J; Joshi, SR; Mohanty, S; Shaw, AC; Lloyd, TE; Brown, GW; Ko, MS; Gorospe, M; Zou, S; Wang, W
      Nature neuroscience 16 1238-47 2013

      Mostrar Resumo
      23912945 23912945
      mRNA and protein expression for novel GABAA receptors θ and ρ2 are altered in schizophrenia and mood disorders; relevance to FMRP-mGluR5 signaling pathway.
      Fatemi, SH; Folsom, TD; Rooney, RJ; Thuras, PD
      Translational psychiatry 3 e271 2013

      Mostrar Resumo
      23778581 23778581
      Inhibition of GSK3β improves hippocampus-dependent learning and rescues neurogenesis in a mouse model of fragile X syndrome.
      Guo, W; Murthy, AC; Zhang, L; Johnson, EB; Schaller, EG; Allan, AM; Zhao, X
      Human molecular genetics 21 681-91 2012

      Mostrar Resumo
      Western Blotting22048960 22048960
      Efficient detection of RNA-protein interactions using tethered RNAs.
      Iioka, H; Loiselle, D; Haystead, TA; Macara, IG
      Nucleic acids research 39 e53 2011

      Mostrar Resumo
      21300640 21300640

      Brochura

      Título
      RNA-Binding Protein Immunoprecipitation

      Ficha de dados

      Título
      Anti-Fragile X Mental Retardation Protein, clone 1C3 - Data Sheet