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MABN1790 Anti-C9ORF72/C9RANT (poly-AP) Antibody, clone 14E2

Anti-C9ORF72 Antibody, clone 14E2 is an antibody against C9RANT (poly-AP) for use in Western Blotting, Immunocytochemistry.

MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Research Sub-Category: Neurodegenerative Diseases Gene Symbol: C9orf72, ALSFTD, FTDALS UniProt Number: Q96LT7
MABN1790
100 μg  
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Key Spec Table

Species ReactivityKey ApplicationsHostFormatAntibody Type
HWB, ICCRPurifiedMonoclonal Antibody
Description
Catalogue NumberMABN1790
DescriptionAnti-C9ORF72/C9RANT (poly-AP) Antibody, clone 14E2
Alternate Names
  • C9ORF72/C9RANT (poly-PA)
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 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. Immunofluorescence localization of exogenously epxressed DPR-GFP fusions in HEK293 transfectants showed dot-like or star-shaped poly-GA inclusions with predominant cytoplasmic and occasional nuclear localization, while poly-GR and poly-PR showed large dot-like intranuclear inclusions and occasional smaller cytoplasmic inclusions. In contrast, poly-PA was evenly distributed throughout the nucleus and cytoplasm without apparent aggregation.
Product Information
FormatPurified
PresentationPurified rat monoclonal IgG1κ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-C9ORF72 Antibody, clone 14E2 is an antibody against C9RANT (poly-AP) for use in Western Blotting, Immunocytochemistry.
Key Applications
  • Western Blotting
  • Immunocytochemistry
Application NotesImmunocytochemistry Analysis: A representative lot detected the expression an exogenously transfected PA175-myc dipeptide repeat (DPR) construct containing a 175 PA-repeat sequence by fluorescent immunocytochemistry staining of 4% paraformaldehyde/4% sucrose-fixed, 0.3% Triton X-100-permeablized HEK293 transfectants (May, S., et al. (2014). Acta Neuropathol. 128(4):485-503).
Western Blotting Analysis: A representative lot detected the expression of an exogenously transfected PA175-myc dipeptide repeat (DPR) construct containing a 175 PA-repeat sequence in lysate from HEK293 transfectants (May, S., et al. (2014). Acta Neuropathol. 128(4):485-503).
Biological Information
ImmunogenRecombinant GST fusion with a 10 AP-repeat sequence (GST-AP10).
Epitopepoly-AP (poly-PA).
Clone14E2
ConcentrationPlease refer to lot specific datasheet.
HostRat
SpecificityClone 14E2 recognizes C9ORF72/C9RANT (poly-AP) and other proteins containing poly-AP (poly-PA) sequence.
IsotypeIgG1κ
Species Reactivity
  • Human
Species Reactivity NoteHuman. Target sequence is not species-specific.
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Gene Symbol
  • C9orf72
  • ALSFTD
  • FTDALS
Purification MethodProtein G Purified
UniProt Number
Molecular WeightVariable. Uncharacterized band(s) may appear in some lysates.
Product Usage Statements
Quality AssuranceEvaluated by Western Blotting iof recombinant GST fusion with a 10 AP-repeat sequence (GST-AP10).

Western Blotting Analysis: 0.5 µg/mL of this antibody detected 3 µg of recombinant GST fusion with a 10 AP-repeat sequence (GST-AP10).
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 μg
Global Trade Item Number
Catalogue Number GTIN
MABN1790 04055977350357