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MABN889-AF488 Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor® 488 Conjugate

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.

MABN889-AF488
100 µL  
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Overview

Replacement Information
Description
Catalogue NumberMABN889-AF488
DescriptionAnti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor® 488 Conjugate
Alternate Names
  • C9ORF72/C9RANT (poly-GA)
  • Protein C9orf72
Background InformationExpansion 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.
References
Product Information
FormatAlexaFluor®488
PresentationPurified mouse monoclonal antibody IgG1 in PBS with 1.5% BSA and 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-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.
Key Applications
  • Immunohistochemistry
  • Western Blotting
Application NotesImmunohistochemistry 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).
Biological Information
ImmunogenPolyethylene glycol conjugated linear peptide containng repeat of 10 Gly-Ala repeats.
Clone5E9
ConcentrationPlease refer to lot specific datasheet.
HostMouse
SpecificityClone 5E9 recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence.
IsotypeIgG1κ
Species Reactivity
  • Human
Species Reactivity NoteHuman. Predicted to react with all species based on 100% sequence homology.
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Purification MethodProtein G purified
UniProt Number
Molecular Weight~54 kDa observed. Uncharacterized bands may be observed in some lysate(s).
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated 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.
Usage Statement
  • 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.
Storage and Shipping Information
Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size100 µL
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Catalogue Number GTIN
MABN889-AF488 04054839253164

Documentation

Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor® 488 Conjugate Certificates of Analysis

TitleLot Number
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate - 3153918 3153918
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate - 3426946 3426946
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate - 3712477 3712477
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate - 3872435 3872435
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate - 3917389 3917389
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor® 488 Conjugate Monoclonal Antibody Q2889115

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