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Anti-C9ORF72/C9RANT (poly-AP), clone 14E2 (rat monoclonal)

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货号：MABN1790

品牌：Millipore 密理博

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重要规格表

Species Reactivity Key Applications Host Format Antibody Type
H WB, ICC R Purified Monoclonal Antibody
Description
Catalogue Number MABN1790
Description Anti-C9ORF72/C9RANT (poly-AP) Antibody, clone 14E2
Alternate Names
  • C9ORF72/C9RANT (poly-PA)
Background Information 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 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.
References
Product Information
Format Purified
Presentation Purified rat monoclonal IgG1κ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Applications
Application Anti-C9ORF72 Antibody, clone 14E2 is an antibody against C9RANT (poly-AP) for use in Western Blotting, Immunocytochemistry.
Key Applications
  • Western Blotting
  • Immunocytochemistry
Application Notes Immunocytochemistry 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
Immunogen Recombinant GST fusion with a 10 AP-repeat sequence (GST-AP10).
Epitope poly-AP (poly-PA).
Clone 14E2
Concentration Please refer to lot specific datasheet.
Host Rat
Specificity Clone 14E2 recognizes C9ORF72/C9RANT (poly-AP) and other proteins containing poly-AP (poly-PA) sequence.
Isotype IgG1κ
Species Reactivity
  • Human
Species Reactivity Note Human. Target sequence is not species-specific.
Antibody Type Monoclonal Antibody
Entrez Gene Number
Gene Symbol
  • C9orf72
  • ALSFTD
  • FTDALS
Purification Method Protein G Purified
UniProt Number
Molecular Weight Variable. Uncharacterized band(s) may appear in some lysates.
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality Assurance Evaluated 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 Conditions Stable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size 100 μg
Transport Information
Supplemental Information
Specifications

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