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MABN778

Sigma-Aldrich

Anti-C9ORF72/C9RANT (poly-GR) Antibody, clone 5A2

clone 5A2, 1 mg/mL, from rat

Sinonimo/i:

c9orf72

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About This Item

Codice UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41

1.620,00 SGD


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Origine biologica

rat

Livello qualitativo

Forma dell’anticorpo

purified antibody

Tipo di anticorpo

primary antibodies

Clone

5A2, monoclonal

Reattività contro le specie

human

Reattività contro le specie (prevista in base all’omologia)

all

Concentrazione

1 mg/mL

tecniche

immunohistochemistry: suitable
western blot: suitable

Isotipo

IgG2aκ

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Questo articolo
MABN1790ABN1356ABN1644
Anti-C9orf72 (short form) from rabbit

ABN1644

Anti-C9orf72 (short form)

Gene Information

human ... C9ORF72(203228)

Gene Information

human ... C9orf72(203228)

Gene Information

human ... C9orf72(203228)

Gene Information

human ... C9orf72(203228)

biological source

rat

biological source

rat

biological source

rabbit

biological source

rabbit

clone

5A2, monoclonal

clone

14E2, monoclonal

clone

polyclonal

clone

polyclonal

antibody form

purified antibody

antibody form

purified immunoglobulin

antibody form

serum

antibody form

affinity isolated antibody

technique(s)

immunohistochemistry: suitable, western blot: suitable

technique(s)

immunocytochemistry: suitable, western blot: suitable

technique(s)

dot blot: suitable, immunofluorescence: suitable, immunohistochemistry: suitable, western blot: suitable

technique(s)

immunofluorescence: suitable, immunohistochemistry: suitable, immunoprecipitation (IP): suitable, western blot: suitable

Descrizione generale

Hexanucleotide (GGGGCC) repeat expansions in a noncoding region of C9ORF72 are the major genetic cause of FTD and ALS (c9FTD/ALS). The RNA structure of GGGGCC repeats renders these transcripts susceptible to an unconventional mechanism of translation—repeat associated non-ATG (RAN) translation. Translation of the GGGGCC-repeat in all reading frames would result in three dipeptide-repeat (DPR) proteins poly-(Gly-Ala), poly-(Gly-Pro) and poly-(Gly-Arg). poly-GA and poly-GP proteins are extremely hydrophobic and may form intracellular aggregates

Specificità

This antibody recognizes C9ORF72/C9RANT (poly-GR) and other proteins containing poly-GR sequence, such as the Epstein Barr virus protein EBNA2

Immunogeno

Epitope: poly-GR
Linear peptide corresponding to Epstein-Barr Virus Nuclear Antigen 2 (ENBA2) containing GR repeat sequence.

Applicazioni

Detect C9ORF72/C9RANT (poly-GR) using this Anti-C9ORF72/C9RANT (poly-GR) antibody, clone 5A2 validated for use in western blotting & IHC.
Immunohistochemistry Analysis: A representative lot detected poly-GR inclusions from brain tissues of C9orf72 patients with C9ORF72 hexanucleotide expansion (Mori, K., et al. (2013). Science. 339(6125):1335-1338.).
Research Category
Neuroscience
Research Sub Category
Neurodegenerative Diseases

Qualità

Evaluated by Western Blotting using GST fusion proteins with 15 GA, GP, or GR repeats, as well as GST alone without any repeat sequence.

Western Blotting Analysis: 0.2 μg of this antibody detected 10 μg of GR-GST recombinant protein with 15 GR repeats.

Descrizione del bersaglio

Variable

Stato fisico

Format: Purified
Protein G Purified
Purified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Stoccaggio e stabilità

Stable for 1 year at 2-8°C from date of receipt.

Esclusione di responsabilità

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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Codice della classe di stoccaggio

12 - Non Combustible Liquids

Classe di pericolosità dell'acqua (WGK)

WGK 1

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable


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    The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
    Mori, K; Weng, SM; Arzberger, T; May, S; Rentzsch, K; Kremmer, E; Schmid, B; Kretzschmar et al.
    Science (New York, N.Y.) null
    Philip McGoldrick et al.
    Neurology, 90(4), e323-e331 (2017-12-29)
    Suggested C9orf72 disease mechanisms for amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration include C9orf72 haploinsufficiency, G4C2/C4G2 RNA foci, and dipeptide repeat (DPR) proteins translated from the G4C2 expansion; however, the role of small expansions (e.g., 30-90 repeats) is unknown
    Alan S Premasiri et al.
    Frontiers in pharmacology, 11, 569661-569661 (2020-10-06)
    Repeat expansion mutations in the C9ORF72 gene are the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Repeat-associated non-AUG translation of this expansion produces dipeptide repeat proteins (DRPs). The arginine containing DRPs, polyGR and polyPR
    Amrutha Swaminathan et al.
    Human molecular genetics, 27(10), 1754-1762 (2018-03-13)
    Large expansions of hexanucleotide GGGGCC (G4C2) repeats (hundreds to thousands) in the first intron of the chromosome 9 open reading frame 72 (C9orf72) locus are the strongest known genetic factor associated with amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Different
    Yingxiao Shi et al.
    JCI insight, 5 (2019-07-17)
    Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease with diverse etiologies. Therefore, the identification of common disease mechanisms and therapeutics targeting these mechanisms could dramatically improve clinical outcomes. To this end, we developed induced motor neuron (iMN) models

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