MABN889-AF488

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

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

• 同義詞: C9ORF72/C9RANT (poly-GA), Protein C9orf72
• 分類程式碼代碼: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

屬性

• 生物源: mouse
• 品質等級: 100
• 共軛: ALEXA FLUOR^™ 488
• 抗體表格: purified immunoglobulin
• 抗體產品種類: primary antibodies
• 無性繁殖: 5E9, monoclonal
• 物種活性: human
• 物種活性（以同源性預測）: all (based on 100% sequence homology)
• 技術: immunohistochemistry: suitable; western blot: suitable
• 同型: IgG1κ
• NCBI登錄號: NP_001242983
• UniProt登錄號: Q96LT7
• 運輸包裝: ambient
• 目標翻譯後修改: unmodified
• 基因資訊: human ... C9ORF72(203228)

描述

一般說明

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.

特異性

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

免疫原

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

應用

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

品質

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.

標靶描述

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

外觀

Protein G purified

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

儲存和穩定性

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

其他說明

Concentration: Please refer to lot specific datasheet.

法律資訊

ALEXA FLUOR is a trademark of Life Technologies

免責聲明

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.

安全資訊

• 儲存類別代碼: 12 - Non Combustible Liquids
• 水污染物質分類（WGK）: WGK 2