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MABN889 Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9

Detect C9ORF72/C9RANT using this Anti-C9ORF72/C9RANT, clone 5E9 Antibody validated for use in Western Blotting and Immunohistochemistry.

MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Research Sub-Category: Developmental Signaling Gene Symbol: C9orf72 UniProt Number: Q96LT7
MABN889
50 µL  
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Key Spec Table

Species ReactivityKey ApplicationsHostFormatAntibody Type
HIHC, WBMPurifiedMonoclonal Antibody
Description
Catalogue NumberMABN889
DescriptionAnti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9
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
FormatPurified
PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationDetect C9ORF72/C9RANT using this Anti-C9ORF72/C9RANT, clone 5E9 Antibody validated for use in Western Blotting and Immunohistochemistry.
Key Applications
  • Immunohistochemistry
  • Western Blotting
Application NotesWestern 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).
Biological Information
ImmunogenPolyethylene glycol-conjugated linear peptide containing poly-GA sequence.
EpitopePoly-GA
Clone5E9
ConcentrationPlease refer to lot specific datasheet.
HostMouse
SpecificityThis antibody 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
Antibody TypeMonoclonal Antibody
Gene Symbol
  • C9orf72
Purification MethodProtein G Purified
UniProt Number
Molecular WeightVariable. Additional uncharacterized bands maybe observed in some lysates.
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated 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
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 Size50 µL
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Catalogue Number GTIN
MABN889 04055977171426

Documentation

Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 SDS

Title

Safety Data Sheet (SDS) 

Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Certificates of Analysis

TitleLot Number
Anti-C9ORF72/C9RANT (poly-GA), -2653164 2653164
Anti-C9ORF72/C9RANT (poly-GA), -2794074 2794074
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3150354 3150354
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3226534 3226534
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3328868 3328868
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3470729 3470729
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3557714 3557714
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3694727 3694727
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3738412 3738412
Anti-C9ORF72/C9RANT (poly-GA), clone 5E9 - 3808623 3808623
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