MABN889-AF488
Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor™ 488 Conjugate
clone 5E9, from mouse, ALEXA FLUOR™ 488
Synonym(s):
C9ORF72/C9RANT (poly-GA), Protein C9orf72
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About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
biological source
mouse
Quality Level
conjugate
ALEXA FLUOR™ 488
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
5E9, monoclonal
species reactivity
human
species reactivity (predicted by homology)
all (based on 100% sequence homology)
technique(s)
immunohistochemistry: suitable
western blot: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
ambient
target post-translational modification
unmodified
Gene Information
human ... C9ORF72(203228)
General description
Expansion of a GGGGCC 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 generates three polypeptides, each composed of repeating units of two amino acids (dipeptide repeats, DPRs), glycine-alanine (GA), glycine-proline (GP), and glycine-arginine (GR), respectively. These DPR proteins form inclusions particularly in the cerebellar cortex, hippocampus and cerebral neocortex. These inclusions are also immunoreactive for markers of the ubiquitin proteasome system (UPS), including ubiquitin, ubiquilins, and p62, but is distinct from inclusions containing TDP-43 that is also found in FTLD-TDP (frontotemporal lobar degeneration with TDP-43 pathology) and ALS cases. Antibodies against poly-GA, poly-GP, and poly-GR sequence are useful for characterizing the neuroanatomical distribution and clinico-pathological association of DPR pathology with C9ORF72 mutations.
Specificity
Clone 5E9 recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence.
Immunogen
Polyethylene glycol conjugated linear peptide containng repeat of 10 Gly-Ala repeats.
Application
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor™ 488 Conjugate, Cat. No. MABN889-AF488, is a highly specific mouse monoclonal antibody that targets Gly-Ala repeats (C9orf72) and has been tested in Immunohistochemistry and Western Blotting.
Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Immunohistochemistry applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).
Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Western Blotting applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).
Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Western Blotting applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).
Research Category
Neuroscience
Neuroscience
Quality
Evaluated by Western Blotting in C9ORF72/C9RANT (poly-GA) GST fusion recombinant 15 GA repeats.
Western Blotting Analysis: 4 µg/mL of this antibody detected Gly-Ala repeats in GST-fusion recombinant C9orf72 protein.
Western Blotting Analysis: 4 µg/mL of this antibody detected Gly-Ala repeats in GST-fusion recombinant C9orf72 protein.
Target description
~54 kDa observed. Uncharacterized bands may be observed in some lysate(s).
Physical form
Protein G purified
Purified mouse monoclonal antibody IgG1 in PBS with 1.5% BSA and 0.05% sodium azide.
Storage and Stability
Stable for 1 year at 2-8°C from date of receipt.
Other Notes
Concentration: Please refer to lot specific datasheet.
Legal Information
ALEXA FLUOR is a trademark of Life Technologies
Disclaimer
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.
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Storage Class Code
12 - Non Combustible Liquids
WGK
WGK 2
Certificates of Analysis (COA)
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