ABN1358

Anti-C9ORF72/C9RANT (Poly-GP sense/antisense)

serum, from rabbit

• 别名: C9ORF72/C9RANT (poly-GP), alpha-GP-sense/antisense
• 分類程式碼代碼: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

属性

• 生物源: rabbit
• 抗體表格: serum
• 抗體產品種類: primary antibodies
• 無性繁殖: polyclonal
• 物種活性: human
• 技術: immunocytochemistry: suitable; immunofluorescence: suitable; immunohistochemistry: suitable; western blot: suitable
• NCBI登錄號: NP_060795
• 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)

说明

一般說明

Expansion of a GGGGCC (G4C2) 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. The sense transcript (5′-GGGGCC-3′ hexanucleotide repeats) is translated through repeat-associated non-ATG (RAN) translation such that poly- (Gly- Ala), poly-(Gly-Pro), and poly-(Gly-Arg) proteins were produced. The anti-sense transcript (containing 5′-GGCCCC-3′ (G2C4) hexanucleotide repeats is translated through repeat-associated non-ATG (RAN) translation producing poly-(Pro-Ala), poly- (Pro- Arg), poly- (Gly- Pro) proteins. RAN proteins with the GP repeat motif are predicted in both the sense and antisense directions (GPsense and GPantisense)The antisense proteins accumulate in cytoplasmic aggregates in affected brain regions, including the frontal and motor cortex, hippocampus, and spinal cord neurons.

特異性

This polyclonal antibody recognizes C9ORF72/C9RANT (poly-GP).

免疫原

H2N-GPGPGPGPGPGPGPGPGCKK-amide.

應用

Anti-C9ORF72/C9RANT (Poly-GP sense/antisense), Cat. No. ABN1358, is a highly specific rabbit polyclonal antibody that targets C9ORF72 ( -GP sense antisense) and has been tested in Immunofluorescence, Immunohistochemistry, Immunocytochemistry, and Western Blotting.

Immunofluorescence Analysis: A representative lot detected C9ORF72/C9RANT (Poly-GP sense/antisense) in transfected HEK293T -V5-GP-Ct cells (Zu, T., et. al. (2013). Proc Natl Acad Sci USE. 110(51):E4968-77).

Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected C9ORF72/C9RANT (Poly-GP sense/antisense) in V5 -GP transfected HEK293. (Courtesy of Dr Laura P.W. Ranum and Dr. Lien Nguyen from the University of Florida).

Western Blotting Analysis: A representative lot detected C9ORF72/C9RANT (Poly-GP sense/antisense) in Western Blotting applications (Zu, T., et. al. (2013). Proc Natl Acad Sci USE. 110(51):E4968-77).

Immunohistochemistry Analysis: A representative lot detected C9ORF72/C9RANT (Poly-GP sense/antisense) in aggregates found in neurons of the CA and DG regions of the hippocampus (Zu, T., et. al. (2013). Proc Natl Acad Sci USE. 110(51):E4968-77).

Research Category
Neuroscience

品質

Evaluated by Western Blotting in transfected HEK293 cell lysate.

Western Blotting Analysis: A 1:1,000 dilution of this antibody detected C9ORF72/C9RANT (Poly-GP sense/antisense) in 10 µL lysate fromHEK293 cells transfected with Flag-GP construct.

標靶描述

~18 kDa observed. Uncharacterized bands may be observed in some lysate(s).

外觀

Unpurified

Rabbit polyclonal antiserum. No preservative.

儲存和穩定性

Stable for 1 year at -20°C from date of receipt. Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

其他說明

Concentration: Please refer to lot specific datasheet.

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.

安全信息

• 儲存類別代碼: 12 - Non Combustible Liquids
• 水污染物質分類（WGK）: WGK 1