MABN778
Anti-C9ORF72/C9RANT (poly-GR) Antibody, clone 5A2
clone 5A2, 1 mg/mL, from rat
Szinonimák:
c9orf72
Bejelentkezésa Szervezeti és Szerződéses árazás megtekintéséhez
Összes fotó(2)
About This Item
UNSPSC kód:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Javasolt termékek
biológiai forrás
rat
Minőségi szint
antitest forma
purified antibody
antitest terméktípus
primary antibodies
klón
5A2, monoclonal
faj reaktivitás
human
faj reaktivitás (homológia által előrejelzett)
all
koncentráció
1 mg/mL
technika/technikák
immunohistochemistry: suitable
western blot: suitable
izotípus
IgG2aκ
kiszállítva
wet ice
célzott transzláció utáni módosítás
unmodified
Géninformáció
human ... C9ORF72(203228)
Általános leírás
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
Egyediség
This antibody recognizes C9ORF72/C9RANT (poly-GR) and other proteins containing poly-GR sequence, such as the Epstein Barr virus protein EBNA2
Immunogen
Epitope: poly-GR
Linear peptide corresponding to Epstein-Barr Virus Nuclear Antigen 2 (ENBA2) containing GR repeat sequence.
Alkalmazás
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
Minőség
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.
Cél megnevezése
Variable
Fizikai forma
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.
Tárolás és stabilitás
Stable for 1 year at 2-8°C from date of receipt.
Jogi nyilatkozat
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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Tárolási osztály kódja
12 - Non Combustible Liquids
WGK
WGK 1
Lobbanási pont (F)
Not applicable
Lobbanási pont (C)
Not applicable
Analitikai tanúsítványok (COA)
Analitikai tanúsítványok (COA) keresése a termék sarzs-/tételszámának megadásával. A sarzs- és tételszámok a termék címkéjén találhatók, a „Lot” vagy „Batch” szavak után.
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Az Ön által nemrégiben megvásárolt termékekre vonatkozó dokumentumokat a Dokumentumtárban találja.
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
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
Amrutha Swaminathan et al.
Human molecular genetics, 27(10), 1754-1762 (2018-03-13)
Large expansions of hexanucleotide GGGGCC (G4C2) repeats (hundreds to thousands) in the first intron of the chromosome 9 open reading frame 72 (C9orf72) locus are the strongest known genetic factor associated with amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Different
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
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
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