FMR1 抗体 (148.1): sc-101048

FMR1 Antibody (148.1) is an IgG₁ κ mouse monoclonal FMR1 antibody (also designated FMRP Translational Regulator 1 antibody, Fragile X Mental Retardation Protein 1 antibody, FRAXA antibody, or Premature Ovarian Failure 1 antibody) suitable for the detection of the FMR1 protein of mouse, human and, to a lesser extent, rat origin by WB, IP and ELISA. FMR1 Antibody (148.1) is available as the non-conjugated anti-FMR1 antibody. Fragile X syndrome is the most frequent form of inherited mental retardation and is the result of transcriptional silencing of the FMR1 gene on the X chromosome. The FMR1 gene contains a distinct CpG dinucleotide repeat located in the 5′ untranslated region of the gene. In fragile X syndrome this tandem repeat is substantially amplified and subjected to extensive methylation and enhanced transcriptional silencing. The FMR1 protein (or FMRP) is an RNA-binding protein that associates with polyribosomes and is a likely component of a messenger ribonuclear protein (mRNP) particle. It contains several features that are characteristics of RNA-binding proteins, including two hnRNPK homology (KH) domains and an RGG amino acid motif (RGG box). FMR1 localizes to both the nucleus and the cytoplasm and can also interact with two fragile X syndrome related factors, FXR1 and FXR2, which form heterodimers through their N-terminal coiled-coil domains. Since FMR1 contains both a nuclear localization signal and a nuclear export signal it is also implicated in the nucleocytoplasmic transport of mRNAs.

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References:

1. Oligomerization properties of fragile-X mental-retardation protein (FMRP) and the fragile-X-related proteins FXR1P and FXR2P.  |  Tamanini, F., et al. 1999. Biochem J. 343 Pt 3: 517-23. PMID: 10527928
2. Isolation of an FMRP-associated messenger ribonucleoprotein particle and identification of nucleolin and the fragile X-related proteins as components of the complex.  |  Ceman, S., et al. 1999. Mol Cell Biol. 19: 7925-32. PMID: 10567518
3. Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome.  |  Verkerk, AJ., et al. 1991. Cell. 65: 905-14. PMID: 1710175
4. Characterization and primary structure of the poly(C)-binding heterogeneous nuclear ribonucleoprotein complex K protein.  |  Matunis, MJ., et al. 1992. Mol Cell Biol. 12: 164-71. PMID: 1729596
5. Absence of expression of the FMR-1 gene in fragile X syndrome.  |  Pieretti, M., et al. 1991. Cell. 66: 817-22. PMID: 1878973
6. The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2.  |  Zhang, Y., et al. 1995. EMBO J. 14: 5358-66. PMID: 7489725
7. A point mutation in the FMR-1 gene associated with fragile X mental retardation.  |  De Boulle, K., et al. 1993. Nat Genet. 3: 31-5. PMID: 8490650
8. The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals.  |  Eberhart, DE., et al. 1996. Hum Mol Genet. 5: 1083-91. PMID: 8842725