Anti-C9ORF72/C9RANT (poly-GP) Antibody

In a noncoding region of C9ORF72 hexanucleotide (GGGGCC) repeat expansions are the major genetic cause of FTD and ALS (c9FTD/ALS). Transcripts that have the RNA structure of GGGGCC repeats are susceptible to an unconventional mechanism of translation called repeatassociated 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. Anti-C9RANT, an antibody that was generated against putative GGGGCC repeat RANtranslated peptides mentioned above, detects insoluble high molecular weight material in brain homogenates, and neuronal inclusions throughout the CNS of c9FTD/ALS cases (Ash, PE, et al. (2013). Neuron. 77(4):639-46.)

Variable

Epitope: poly-GP

Linear peptides corresponding to C-Ahx-(GA)8-amide, C-Ahx-(GP)8-amide, and C-Ahx-
(GR)8-amide

Anti-C9ORF72/C9RANT (poly-GP), Cat # ABN455, is a highly validated rabbit Polyclonal antibody, that targets C9ORF72/C9RANT (poly-GP) and has been tested in Western Blotting and Immunohistochemistry.

Immunohistochemistry Analysis: A 1:5000 dilution from a representative lot detected C9RANT (poly-GP) in human cerebellar sections of c9FTD cases (Ash, PE, et al. (2013). Neuron. 77(4):639-646.).

Research Category
Neuroscience

Neuroscience

Research Sub Category
Neurodegenerative Diseases

Synapse & Synaptic Biology

Format: Unpurified

Rabbit Polyclonal serum with 0.05% sodium azide.

Unpurified

Stable for 1 year at -20°C from date of receipt.
Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Evaluated by Western Blotting in transfected HEK293 cell lysate.

Western Blotting Analysis: 1:2000 diultion of this antibody detected GST fusion protein with 5 GP repeats in 10 µg of transfected HEK293 cell lysate.

Concentration: Please refer to lot specific datasheet.

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.