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Millipore Detect C9orf72/C9rant (Poly-Gr) Using This Anti-C9orf72/C9rant (Poly-Gr) Antibody, Clone 5a2 Validated For Use In Western Blotting & Ihc. - Mill (Additional S&H or Hazmat Fees May Apply)
Hexanucleotide (GGGGCC) repeat expansions in a noncoding region of C9ORF72 are the major genetic cause of FTD and ALS (c9FTD/ALS). The RNA structure of GGGGCC repeats renders these transcripts susceptible to an unconventional mechanism of translation—repeat associated non-ATG (RAN) translation. Translation of the GGGGCC-repeat in all reading frames would result in three dipeptide-repeat (DPR) proteins poly-(Gly-Ala), poly-(Gly-Pro) and poly-(Gly-Arg). poly-GA and poly-GP proteins are extremely hydrophobic and may form intracellular aggregates
Product Information
Format
Purified
Presentation
Purified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality Level
MQ100
Applications
Application
Detect C9ORF72/C9RANT (poly-GR) using this Anti-C9ORF72/C9RANT (poly-GR) antibody, clone 5A2 validated for use in western blotting & IHC.
Key Applications
Western Blotting
Immunohistochemistry
Application Notes
Immunohistochemistry Analysis: A representative lot detected poly-GR inclusions from brain tissues of C9orf72 patients with C9ORF72 hexanucleotide expansion (Mori, K., et al. (2013). Science. 339(6125):1335-1338.).
Biological Information
Immunogen
Linear peptide corresponding to Epstein-Barr Virus Nuclear Antigen 2 (ENBA2) containing GR repeat sequence.
Epitope
poly-GR
Clone
5A2
Concentration
1 mg/mL
Host
Rat
Specificity
This antibody recognizes C9ORF72/C9RANT (poly-GR) and other proteins containing poly-GR sequence, such as the Epstein Barr virus protein EBNA2
Isotype
IgG2aκ
Species Reactivity
Human
Species Reactivity Note
Human. Predicted to react with all species.
Antibody Type
Monoclonal Antibody
Purification Method
Protein G Purified
Molecular Weight
Variable
Product Usage Statements
Quality Assurance
Evaluated by Western Blotting using GST® fusion proteins with 15 GA, GP, or GR repeats, as well as GST alone without any repeat sequence.
Western Blotting Analysis: 0.2 μg of this antibody detected 10 μg of GR-GST recombinant protein with 15 GR repeats.
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.
