Anti-C9ORF72/C9RANT (Poly-GR)

Expansion of a GGGGCC (G4C2) hexanucleotide repeat sequence in the non-coding region of human chromosome 9 open reading frame 72 or C9orf72 (also known as ALSFTD, FTDALS; Gene ID 203228) is the most common genetic abnormality in familial and sporadic frontotemporal dementia (FTD) and motor neuron disease (MND), with amyotrophic lateral sclerosis (ALS) as the most frequent form. The number of hexanucleotide repeats in the normal population ranges from 2 to 24, whereas up to several thousand repeats (700 - 4,400 repeats) are found in the pathologically expanded allele. Unconventional repeat-associated non-ATG-initiated translation (RANT) of the GGGGCC repeats in the three alternate reading frames from both sense and antisense transcripts generates five types of dipeptide repeat (DPR) protein species, each composed of repeating units of two amino acids, glycine-alanine (GA), glycine-proline (GP), glycine-arginine (GR), alanine-proline (AP), proline-arginine (PR), respectively. The sense transcript (5′-GGGGCC-3′ hexanucleotide repeats) is translated through repeat-associated non-ATG (RAN) translation such that poly- (Gly- Ala), poly-(Gly-Pro), and poly-(Gly-Arg) proteins were produced. The anti-sense transcript (containing 5′-GGCCCC-3′ (G2C4) hexanucleotide repeats is translated through repeat-associated non-ATG (RAN) translation producing poly-(Pro-Ala), poly- (Pro- Arg), poly- (Gly- Pro) proteins. RAN proteins with the GP repeat motif are predicted in both the sense and antisense directions (GPsense and GPantisense)The antisense proteins accumulate in cytoplasmic aggregates in affected brain regions, including the frontal and motor cortex, hippocampus, and spinal cord neurons.

~17.5 kDa observed. Uncharacterized bands may be observed in some lysate(s).

Ac-RGRGRGRGRGRGRGRGRC-amide.

Epitope: unknown

Anti-C9ORF72/C9RANT (Poly-GR), Cat. No. ABN1361, is a highly specific rabbit polyclonal antibody that targets C9ORF72 ( -GR sense) and has been tested in Immunofluorescence, Immunohistochemistry, Immunocytochemistry, and Western Blotting.

Research Category
Neuroscience

Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (Poly-GR) in Western Blotting applications (Zu, T., et. al. (2013). Proc Natl Acad Sci USA. 110(51):E4968-77).

Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (Poly-GR) in aggregates found in neurons of the CA and DG regions of the hippocampus (Zu, T., et. al. (2013). Proc Natl Acad Sci USA. 110(51):E4968-77).

Immunofluorescence Analysis: A representative lot detected C9ORF72/C9RANT (Poly-GR) in V5-GR-Ct Transfected HEK293T (Zu, T., et. al. (2013). Proc Natl Acad Sci USA. 110(51):E4968-77).

Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected C9ORF72/C9RANT (Poly-GR) in V5-GR-Ct transfected HEK293. (Courtesy of Dr Laura P.W. Ranum and Dr. Lien Nguyen from the University of Florida).

This polyclonal antibody recognizes C9ORF72/C9RANT (poly-GR).

Rabbit polyclonal antiserum 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: A 1:1,000 dilution of this antibody detected C9ORF72/C9RANT (Poly-GR) in 10 µL of lysate from HEK293 cells transfected with Flag-GR construct.

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.