| Description | |
|---|---|
| Catalogue Number | MABN889-AF488 |
| Description | Anti-C9ORF72/C9RANT (poly-GA) Antibody, clone 5E9 Antibody, Alexa Fluor® 488 Conjugate |
| Alternate Names |
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| 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 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. |
| Product Information | |
|---|---|
| Format | AlexaFluor®488 |
| Presentation | Purified mouse monoclonal antibody IgG1 in PBS with 1.5% BSA and 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
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| 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. |
| Key Applications |
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| Application Notes | 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). |
| Biological Information | |
|---|---|
| Immunogen | Polyethylene glycol conjugated linear peptide containng repeat of 10 Gly-Ala repeats. |
| Clone | 5E9 |
| Concentration | Please refer to lot specific datasheet. |
| Host | Mouse |
| Specificity | Clone 5E9 recognizes C9ORF72/C9RANT (poly-GA) and other proteins containing poly-GA sequence. |
| Isotype | IgG1κ |
| Species Reactivity |
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| Species Reactivity Note | Human. Predicted to react with all species based on 100% sequence homology. |
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Purification Method | Protein g purified |
| UniProt Number |
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| Molecular Weight | ~54 kDa observed. Uncharacterized bands may be observed in some lysate(s). |
| Product Usage Statements | |
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| Quality Assurance | 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. |
| Usage Statement |
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| Storage and Shipping Information | |
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| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
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| Material Size | 100 µL |