MABN1790

Anti-C9ORF72/C9RANT (poly-AP) Antibody, clone 14E2

clone 14E2, from rat

• Synonyme(s) : C9ORF72/C9RANT (poly-PA)
• Code UNSPSC : 12352203
• eCl@ss : 32160702
• Nomenclature NACRES : NA.43

Propriétés

• Source biologique: rat
• Niveau de qualité: 100
• Forme d'anticorps: purified immunoglobulin
• Type de produit anticorps: primary antibodies
• Clone: 14E2, monoclonal
• Espèces réactives: human
• Technique(s): immunocytochemistry: suitable; western blot: suitable
• Isotype: IgG1κ
• Numéro d'accès UniProt: Q96LT7
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... C9orf72(203228)

Description

Description générale

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.

Spécificité

Clone 14E2 recognizes C9ORF72/C9RANT (poly-AP) and other proteins containing poly-AP (poly-PA) sequence.

Target sequence is not species-specific.

Immunogène

Epitope: poly-AP (poly-PA).

Recombinant GST fusion with a 10 AP-repeat sequence (GST-AP10).

Application

Anti-C9ORF72 Antibody, clone 14E2 is an antibody against C9RANT (poly-AP) for use in Western Blotting, Immunocytochemistry.

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).

Qualité

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).

Description de la cible

Variable. Uncharacterized band(s) may appear in some lysates.

Forme physique

Format: Purified

Autres remarques

Concentration: Please refer to lot specific datasheet.

Informations sur la sécurité

• Code de la classe de stockage: 12 - Non Combustible Liquids
• Classe de danger pour l'eau (WGK): WGK 1
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable