MABN889-AF488

Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor^™ 488 Conjugate

clone 5E9, from mouse, ALEXA FLUOR^™ 488

• Synonym(s): C9ORF72/C9RANT (poly-GA), Protein C9orf72
• UNSPSC Code: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

Properties

• biological source: mouse
• Quality Level: 100
• conjugate: ALEXA FLUOR^™ 488
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: 5E9, monoclonal
• species reactivity: human
• species reactivity (predicted by homology): all (based on 100% sequence homology)
• technique(s): immunohistochemistry: suitable; western blot: suitable
• isotype: IgG1κ
• NCBI accession no.: NP_001242983
• UniProt accession no.: Q96LT7
• shipped in: ambient
• target post-translational modification: unmodified
• Gene Information: human ... C9ORF72(203228)

Description

General description

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.

Specificity

Clone 5E9 recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence.

Immunogen

Polyethylene glycol conjugated linear peptide containng repeat of 10 Gly-Ala repeats.

Application

Anti-C9ORF72/C9RANT (poly-GA), clone 5E9, Alexa Fluor^™ 488 Conjugate, Cat. No. MABN889-AF488, is a highly specific mouse monoclonal antibody that targets Gly-Ala repeats (C9orf72) and has been tested in Immunohistochemistry and Western Blotting.

Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Immunohistochemistry applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).

Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (poly-GA) Western Blotting applications (Mackenzie, I.R., et. al. (2013). Acta Neuropathol. 126(6):859-79).

Research Category
Neuroscience

Quality

Evaluated by Western Blotting in C9ORF72/C9RANT (poly-GA) GST fusion recombinant 15 GA repeats.

Western Blotting Analysis: 4 µg/mL of this antibody detected Gly-Ala repeats in GST-fusion recombinant C9orf72 protein.

Target description

~54 kDa observed. Uncharacterized bands may be observed in some lysate(s).

Physical form

Protein G purified

Purified mouse monoclonal antibody IgG1 in PBS with 1.5% BSA and 0.05% sodium azide.

Storage and Stability

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

Other Notes

Concentration: Please refer to lot specific datasheet.

Legal Information

ALEXA FLUOR is a trademark of Life Technologies

Disclaimer

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.

Safety Information

• Storage Class Code: 12 - Non Combustible Liquids
• WGK: WGK 2