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Key Specifications Table
| 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 |
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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 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. |
| 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. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-C9ORF72 Antibody, clone 14E2 is an antibody against C9RANT (poly-AP) for use in Western Blotting, Immunocytochemistry. |
| Key Applications |
|
| 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 |
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| Species Reactivity Note | Human. Target sequence is not species-specific. |
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Gene Symbol |
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| Purification Method | Protein G Purified |
| UniProt Number |
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| Molecular Weight | Variable. Uncharacterized band(s) may appear in some lysates. |
| 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 |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 μg |