Fragile X/FMRP Signaling Pathway Antibody Sampler Kit #48267
Product Information
Kit Usage Information
Protocols
- 2331: Western Blotting
- 2332: Western Blotting, Immunofluorescence
- 7074: Western Blotting
- 7098: Western Blotting, Immunofluorescence
- 7104: Western Blotting, Immunofluorescence
- 12295: Western Blotting, Immunofluorescence
- 12551: Western Blotting, Immunoprecipitation (Agarose), Immunohistochemistry (Paraffin), Immunofluorescence
- 44353: Western Blotting
- 55920: Western Blotting, Immunoprecipitation (Agarose), Immunofluorescence
Product Description
Specificity / Sensitivity
Each antibody in the Fragile X/FMRP Signaling Pathway Antibody Sampler Kit detects endogenous levels of its target protein. Phospho-eEF2 (Thr56) Antibody detects endogenous levels of eEF2 only when phosphorylated at Thr56. It does not recognize eEF2 phosphorylated at other sites.
Source / Purification
Monoclonal antibodies are produced by immunizing rabbits with synthetic peptides corresponding to Gly552 of human FMRP protein, Leu1105 of human mGluR1 protein, Gly574 of human FXR1 protein, and Gly477 of human FXR2 protein. mGluR5 (D6E7B) Rabbit mAb is produced by immunizing animals with recombinant protein specific to the carboxy terminus of human mGluR5 protein. eEF2 Antibody and CYFIP1 Antibody are produced by immunizing animals with a synthetic peptide corresponding to residues at the amino-terminus of human eEF2 and residues surrounding Pro527 of human CYFIP1 protein, respectively. Phospho-eEF2 (Thr56) Antibody is produced by immunizing animals with a synthetic phosphopeptide corresponding to residues surrounding Thr56 of human eEF2. Antibodies are purified by protein A and peptide affinity chromatography.
Background
Fragile X syndrome, a frequent cause of inherited mental retardation, often results from expansion of the CGG trinucleotide repeat in the gene that encodes the fragile X mental retardation protein (FMRP, [1]). FMRP (also known as FMR1) and its two autosomal homologs (FXR1 and FXR2) all bind RNA and play a role in the pathogenesis of fragile X syndrome (1-3). Each of these related proteins can associate with one another as well as form homodimers and complexes with other RNA-binding proteins like cytoplasmic FMRP interacting protein 1 (CYFIP1, [3,4]). FMRP, FXR1, FXR2, and CYFIP1 have been implicated in the translational regulation of mRNAs (5,6). Importantly, this complex of proteins may be dynamically regulated to drive protein synthesis-dependent forms of synaptic plasticity in response to specific activity. That is, activation of metabotropic glutamate receptors, including mGluR1 and mGlur5, can regulate FMRP-dependent forms of translation via post-translational modification of eukaryotic elongation factor 2 (eEF2) to locally control dynamic translation of important synaptic proteins, which, subsequently, alter synaptic function (7-9).
- Verkerk, A.J. et al. (1991) Cell 65, 905-14.
- Siomi, M.C. et al. (1995) EMBO J 14, 2401-8.
- Zhang, Y. et al. (1995) EMBO J 14, 5358-66.
- Abekhoukh, S. et al. (2017) Dis Model Mech 10, 463-74.
- Linder, B. et al. (2008) Hum Mol Genet 17, 3236-46.
- De Rubeis, S. et al. (2013) Neuron 79, 1169-82.
- Park, S. et al. (2008) Neuron 59, 70-83.
- Barnes, S.A. et al. (2015) J Neurosci 35, 15073-81.
- Paul, A. et al. (2019) Front Mol Neurosci 12, 97.
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