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05-1235 | Anti-FMR1 Antibody, clone 7H3.2

100 µL  
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      Replacement Information

      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H WB, ICC M Purified Monoclonal Antibody
      Catalogue Number05-1235
      DescriptionAnti-FMR1 Antibody, clone 7H3.2
      Alternate Names
      • Fragile X mental retardation 1 protein
      • FMRP
      • Protein FMR-1
      Background InformationFragile X Mental Retardation 1 (FMR1) is a RNA-binding protein that is associated to polysomes. Studies suggest this protein is 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.
      Product Information
      • Raji cell lysate
      PresentationPurified mouse monoclonal IgMκ in buffer containing PBS with 0.05% sodium azide.
      ApplicationAnti-FMR1 Antibody, clone 7H3.2 is a Mouse Monoclonal Antibody for detection of FMR1 also known as Fragile X mental retardation 1 protein, FMRP & has been validated in WB, ICC.
      Key Applications
      • Western Blotting
      • Immunocytochemistry
      Application NotesImmunocytochemistry Analysis: 1:250 dilution from a representative lot detected FMR1 in HeLa and A431 cells.
      Biological Information
      ImmunogenLinear peptide corresponding to human FMR1.
      Species Reactivity
      • Human
      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. Expansion of the trinucleotide repeat may also cause one form of premature ovarian failure (POF1). Multiple alternatively spliced transcript variants that encode different protein isoforms and which are located in different cellular locations have been described for this gene. [provided by RefSeq].
      Gene Symbol
      • FMR1
      Purification MethodIon Exchange
      UniProt Number
      UniProt SummaryFUNCTION: Translation repressor. Component of the CYFIP1-EIF4E-FMR1 complex which binds to the mRNA cap and mediates translational repression. In the CYFIP1-EIF4E-FMR1 complex this subunit mediates translation repression (By similarity). RNA-binding protein that plays a role in intracellular RNA transport and in the regulation of translation of target mRNAs. Associated with polysomes. May play a role 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).

      SUBUNIT STRUCTURE: Component of the CYFIP1-EIF4E-FMR1 complex which is composed of CYFIP, EIF4E and FMR1. Interacts with CYFIP1 and CYFIP2. The interaction with brain cytoplasmic RNA 1 (BC1) increases binding affinity for the CYFIP1-EIF4E complex in the brain (By similarity). Homooligomer. Found in a RNP granule complex with IGF2BP1. Directly interacts with SMN and TDRD3. Interacts with the SMN core complex that contains SMN1, SIP1/GEMIN2, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts with FXR1, FXR2, IGF2BP1, NUFIP1, NUFIP2, MCRS1 and RANBP9.

      SUBCELLULAR LOCATION: Cytoplasm. Nucleus › nucleolus

      TISSUE SPECIFICTY: Highest levels found in neurons, brain, testis, placenta and lymphocytes. Also expressed in epithelial tissues and at very low levels in glial cells.

      PTM: Phosphorylated on several serine residues (By similarity).

      INVOLVEMENT IN DISEASE: Defects in FMR1 are the cause of fragile X syndrome (FRAX) [MIM:300624]. Fragile X syndrome 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.

      Defects in FMR1 are the cause of premature ovarian failure syndrome type 1 (POF1) [MIM:311360]. An ovarian disorder defined as the cessation of ovarian function under the age of 40 years. It is characterized by oligomenorrhea or amenorrhea, in the presence of elevated levels of serum gonadotropins and low estradiol.

      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.

      SEQUENCE SIMILARITIES: Belongs to the FMR1 family.

      Contains 2 KH domains.

      SEQUENCE CAUTION: The sequence AAA52458.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

      The sequence AAA62466.1 differs from that shown. Reason: Erroneous gene model prediction.

      The sequence AAA62467.1 differs from that shown. Reason: Erroneous gene model prediction.
      Molecular Weight~71 kDa observed. This protein has 8 reported isoforms ranging from 66-71 kDa.
      Physicochemical Information
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot in Raji cell lysate.

      Western Blot Analysis: 1:50,000 dilution of this antibody detected FMR1 on 10 µg of Raji cell lysate.
      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 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information




      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-FMR1, clone 7H3.2 - 2420513 2420513
      Anti-FMR1, clone 7H3.2 - 2512960 2512960
      Anti-FMR1, clone 7H3.2 - NRG1875749 NRG1875749
      Anti-FMR1, clone 7H3.2 -2621512 2621512
      Anti-FMR1, clone 7H3.2 -2730004 2730004


      Reference overviewPub Med ID
      Negative regulation of interleukin 2 transcription by the glucocorticoid receptor.
      J P Northrop,G R Crabtree,P S Mattila
      The Journal of experimental medicine 175 1992

      Show Abstract Full Text Article
      1569395 1569395

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      Life Science Research > Antibodies and Assays > Primary Antibodies