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.
This polyclonal antibody recognizes C9ORF72/C9RANT (GP-C-terminal).
Anti-C9ORF72/C9RANT (GPsense-CT), Cat. No. ABN1357, is a highly specific rabbit polyclonal antibody that targets C9ORF72 ( -GP sense, CT) and has been tested in Immunocytochemistry, Immunofluorescence, Immunohistochemistry, and Western Blotting.
Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected C9ORF72/C9RANT (GPsense-CT) in V5-(GP)120-Ct transfected HEK293. (Courtesy of Dr Laura P.W. Ranum and Dr. Lien Nguyen from the University of Florida).
Immunofluorescence Analysis: A representative lot detected C9ORF72/C9RANT (GPsense-CT) in V5-GP-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 (GPsense-CT) in HEK293 cells.
Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (GPsense-CT) 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).
Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (GPsense-CT) in Western Blotting applications (Zu, T., et. al. (2013). Proc Natl Acad Sci USA. 110(51):E4968-77).
Evaluated by Western Blotting in transfected HEK293 cell lysate.
Western Blotting Analysis: A 1:1,000 dilution of this antibody detected C9ORF72/C9RANT (GPsense-CT) in 10 µL lysate from HEK293 cells transfected with V5-GP-Ct construct.
~60 kDa observed. Uncharacterized bands may be observed in some lysate(s).
Concentration: Please refer to lot specific datasheet.
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