MABN889
Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9
clone 5E9, from mouse
Synonym(s):
C9ORF72/C9RANT (poly-GA), Protein C9orf72
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About This Item
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
biological source
mouse
Quality Level
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
5E9, monoclonal
species reactivity
human
species reactivity (predicted by homology)
all
technique(s)
immunohistochemistry: suitable
western blot: suitable
isotype
IgG1κ
UniProt accession no.
shipped in
wet ice
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
This antibody recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence.
Immunogen
Epitope: Poly-GA
Polyethylene glycol-conjugated linear peptide containing poly-GA sequence.
Application
Detect C9ORF72/C9RANT using this Anti-C9ORF72/C9RANT, clone 5E9 Antibody validated for use in Western Blotting and Immunohistochemistry.
Research Category
Neuroscience
Neuroscience
Research Sub Category
Developmental Signaling
Developmental Signaling
Western Blotting Analysis: A representative lot detected recombinant GST fusion with 15 GA repeats (Mackenzie, I.R., et al. (2013). Acta Neuropahol. 126(6):859-879).
Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT with 15 GA repeats in cerebellum from a C9ORF72 mutation case (Mackenzie, I.R., et al. (2013). Acta Neuropahol. 126(6):859-879).
Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT with 15 GA repeats in cerebellum from a C9ORF72 mutation case (Mackenzie, I.R., et al. (2013). Acta Neuropahol. 126(6):859-879).
Quality
Evaluated by Western blotting using GST fusion recombinant protein with 15 GA repeats.
Western Blotting Analysis: 0.05 µg/mL of this antibody detected 10 ug of GST fusion recombinant protein with 15 GA repeats
Western Blotting Analysis: 0.05 µg/mL of this antibody detected 10 ug of GST fusion recombinant protein with 15 GA repeats
Target description
Variable. Additional uncharacterized bands maybe observed in some lysates.
Physical form
Format: Purified
Protein G Purified
Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 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.
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 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.
Youn-Bok Lee et al.
Human molecular genetics, 30(3-4), 318-320 (2020-09-06)
Daniel A Solomon et al.
Brain : a journal of neurology, 141(10), 2908-2924 (2018-09-22)
Accumulation and aggregation of TDP-43 is a major pathological hallmark of amyotrophic lateral sclerosis and frontotemporal dementia. TDP-43 inclusions also characterize patients with GGGGCC (G4C2) hexanucleotide repeat expansion in C9orf72 that causes the most common genetic form of amyotrophic lateral
Annelies Quaegebeur et al.
Acta neuropathologica communications, 8(1), 184-184 (2020-11-11)
A C9orf72 repeat expansion is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis. One of the suggested pathomechanisms is toxicity from dipeptide repeat proteins (DPRs), which are generated via unconventional translation of sense and antisense
Feilin Liu et al.
Acta neuropathologica communications, 10(1), 22-22 (2022-02-16)
The most common inherited cause of two genetically and clinico-pathologically overlapping neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), is the presence of expanded GGGGCC intronic hexanucleotide repeats in the C9orf72 gene. Aside from haploinsufficiency and toxic RNA
Zachary T McEachin et al.
Neuron, 107(2), 292-305 (2020-05-07)
GGGGCC hexanucleotide repeat expansions (HREs) in C9orf72 cause amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) and lead to the production of aggregating dipeptide repeat proteins (DPRs) via repeat associated non-AUG (RAN) translation. Here, we show the similar intronic GGCCTG HREs
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