MABN889
Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9
clone 5E9, from mouse
Sinônimo(s):
C9ORF72/C9RANT (poly-GA), Protein C9orf72
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About This Item
Código UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Produtos recomendados
fonte biológica
mouse
Nível de qualidade
forma do anticorpo
purified immunoglobulin
tipo de produto de anticorpo
primary antibodies
clone
5E9, monoclonal
reatividade de espécies
human
reatividade da espécie (prevista por homologia)
all
técnica(s)
immunohistochemistry: suitable
western blot: suitable
Isotipo
IgG1κ
nº de adesão UniProt
Condições de expedição
wet ice
modificação pós-traducional do alvo
unmodified
Informações sobre genes
human ... C9ORF72(203228)
Descrição geral
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 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.
Especificidade
This antibody recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence.
Imunogênio
Epitope: Poly-GA
Polyethylene glycol-conjugated linear peptide containing poly-GA sequence.
Aplicação
Detect C9ORF72/C9RANT using this Anti-C9ORF72/C9RANT, clone 5E9 Antibody validated for use in Western Blotting and Immunohistochemistry.
Research Category
Neuroscience
Neuroscience
Research Sub Category
Developmental Signaling
Developmental Signaling
Western 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).
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).
Qualidade
Evaluated 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
Western Blotting Analysis: 0.05 µg/mL of this antibody detected 10 ug of GST fusion recombinant protein with 15 GA repeats
Descrição-alvo
Variable. Additional uncharacterized bands maybe observed in some lysates.
forma física
Format: Purified
Protein G Purified
Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Armazenamento e estabilidade
Stable for 1 year at 2-8°C from date of receipt.
Outras notas
Concentration: Please refer to lot specific datasheet.
Exoneração de responsabilidade
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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Código de classe de armazenamento
12 - Non Combustible Liquids
Classe de risco de água (WGK)
WGK 1
Ponto de fulgor (°F)
Not applicable
Ponto de fulgor (°C)
Not applicable
Certificados de análise (COA)
Busque Certificados de análise (COA) digitando o Número do Lote do produto. Os números de lote e remessa podem ser encontrados no rótulo de um produto após a palavra “Lot” ou “Batch”.
Já possui este produto?
Encontre a documentação dos produtos que você adquiriu recentemente na biblioteca de documentos.
Daniel A Solomon et al.
Brain : a journal of neurology, 141(10), 2908-2924 (2018-09-22)
Accumulation and aggregation of TDP-43 is a major pathological hallmark of amyotrophic lateral sclerosis and frontotemporal dementia. TDP-43 inclusions also characterize patients with GGGGCC (G4C2) hexanucleotide repeat expansion in C9orf72 that causes the most common genetic form of amyotrophic lateral
C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.
Youn-Bok Lee et al.
Human molecular genetics, 30(3-4), 318-320 (2020-09-06)
Annelies Quaegebeur et al.
Acta neuropathologica communications, 8(1), 184-184 (2020-11-11)
A C9orf72 repeat expansion is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis. One of the suggested pathomechanisms is toxicity from dipeptide repeat proteins (DPRs), which are generated via unconventional translation of sense and antisense
Feilin Liu et al.
Acta neuropathologica communications, 10(1), 22-22 (2022-02-16)
The most common inherited cause of two genetically and clinico-pathologically overlapping neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), is the presence of expanded GGGGCC intronic hexanucleotide repeats in the C9orf72 gene. Aside from haploinsufficiency and toxic RNA
Matthew D Cykowski et al.
Journal of neuropathology and experimental neurology, 76(5), 402-413 (2017-05-19)
To determine the significance of TAR DNA binding protein 43 kDa (TDP-43) pathology in amyotrophic lateral sclerosis (ALS), we examined the whole brains and spinal cords of 57 patients (35 men; 22 women; mean age 63.3 years; 15 patients with c9orf72-associated
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