MABN778
Anti-C9ORF72/C9RANT (poly-GR) Antibody, clone 5A2
clone 5A2, 1 mg/mL, from rat
동의어(들):
c9orf72
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크기 선택
제품정보 (DICE 배송 시 비용 별도)
UNSPSC Code:
12352203
NACRES:
NA.41
eCl@ss:
32160702
Clone:
5A2, monoclonal
Species reactivity:
human
Application:
IHC, WB
Citations:
17
biological source
rat
antibody form
purified antibody
antibody product type
primary antibodies
clone
5A2, monoclonal
species reactivity
human
species reactivity (predicted by homology)
all
concentration
1 mg/mL
technique(s)
immunohistochemistry: suitable, western blot: suitable
isotype
IgG2aκ
shipped in
wet ice
target post-translational modification
unmodified
Quality Level
Gene Information
human ... C9ORF72(203228)
General description
Hexanucleotide (GGGGCC) repeat expansions in a noncoding region of C9ORF72 are the major genetic cause of FTD and ALS (c9FTD/ALS). The RNA structure of GGGGCC repeats renders these transcripts susceptible to an unconventional mechanism of translation—repeat associated non-ATG (RAN) translation. Translation of the GGGGCC-repeat in all reading frames would result in three dipeptide-repeat (DPR) proteins poly-(Gly-Ala), poly-(Gly-Pro) and poly-(Gly-Arg). poly-GA and poly-GP proteins are extremely hydrophobic and may form intracellular aggregates
Variable
Immunogen
Epitope: poly-GR
Linear peptide corresponding to Epstein-Barr Virus Nuclear Antigen 2 (ENBA2) containing GR repeat sequence.
Application
Detect C9ORF72/C9RANT (poly-GR) using this Anti-C9ORF72/C9RANT (poly-GR) antibody, clone 5A2 validated for use in western blotting & IHC.
Immunohistochemistry Analysis: A representative lot detected poly-GR inclusions from brain tissues of C9orf72 patients with C9ORF72 hexanucleotide expansion (Mori, K., et al. (2013). Science. 339(6125):1335-1338.).
Research Category
Neuroscience
Neuroscience
Research Sub Category
Neurodegenerative Diseases
Neurodegenerative Diseases
Biochem/physiol Actions
This antibody recognizes C9ORF72/C9RANT (poly-GR) and other proteins containing poly-GR sequence, such as the Epstein Barr virus protein EBNA2
Physical form
Format: Purified
Protein G Purified
Purified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Preparation Note
Stable for 1 year at 2-8°C from date of receipt.
Analysis Note
Evaluated by Western Blotting using GST fusion proteins with 15 GA, GP, or GR repeats, as well as GST alone without any repeat sequence.
Western Blotting Analysis: 0.2 μg of this antibody detected 10 μg of GR-GST recombinant protein with 15 GR repeats.
Western Blotting Analysis: 0.2 μg of this antibody detected 10 μg of GR-GST recombinant protein with 15 GR repeats.
Disclaimer
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.
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저장 등급
12 - Non Combustible Liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
Sulfated disaccharide protects membrane and DNA damages from arginine-rich dipeptide repeats in ALS.
Yu-Jen Chang et al.
Science advances, 10(8), eadj0347-eadj0347 (2024-02-23)
Hexanucleotide repeat expansion in C9ORF72 (C9) is the most prevalent mutation among amyotrophic lateral sclerosis (ALS) patients. The patients carry over ~30 to hundreds or thousands of repeats translated to dipeptide repeats (DPRs) where poly-glycine-arginine (GR) and poly-proline-arginine (PR) are
Jeannie Chew et al.
Molecular neurodegeneration, 14(1), 9-9 (2019-02-16)
A G4C2 hexanucleotide repeat expansion in the noncoding region of C9orf72 is the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (c9FTD/ALS). Putative disease mechanisms underlying c9FTD/ALS include toxicity from sense G4C2 and antisense G2C4 repeat-containing RNA, and
Joni Vanneste et al.
Scientific reports, 9(1), 15728-15728 (2019-11-02)
Repeat expansions in the C9orf72 gene cause amyotrophic lateral sclerosis and frontotemporal dementia characterized by dipeptide-repeat protein (DPR) inclusions. The toxicity associated with two of these DPRs, poly-GR and poly-PR, has been associated with nucleocytoplasmic transport. To investigate the causal
Alan S Premasiri et al.
Frontiers in pharmacology, 11, 569661-569661 (2020-10-06)
Repeat expansion mutations in the C9ORF72 gene are the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Repeat-associated non-AUG translation of this expansion produces dipeptide repeat proteins (DRPs). The arginine containing DRPs, polyGR and polyPR
Philip McGoldrick et al.
Neurology, 90(4), e323-e331 (2017-12-29)
Suggested C9orf72 disease mechanisms for amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration include C9orf72 haploinsufficiency, G4C2/C4G2 RNA foci, and dipeptide repeat (DPR) proteins translated from the G4C2 expansion; however, the role of small expansions (e.g., 30-90 repeats) is unknown
국제 무역 품목 번호
| SKU | GTIN |
|---|---|
| MABN778 | 04053252947063 |
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