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折扣/货期| 货号：GTX85161-PEP | 规格：100μl | 目录价：￥2400 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
YBX2 Peptide
别名:
C9orf72-SMCR8 complex subunit , ALSFTD , DENNL72 , FTDALS , FTDALS1
产品描述:
C9orf72 antibody detects the C9orf72 protein, which is 481 amino acids in length and has a predicted molecular weight of 54 kDa. The accumulation of abnormal nucleotide repeats in the C9orf72 gene is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), accounting for approximately 34.2% and 25.9% of cases according to van Blitterswijk et al. (PMID: 23160421).
反应种属:
Human, Mouse, Rat
宿主来源:
Mouse
实验应用:
ICC/IF, IHC-P, WB
靶标/特异性:
KO/KD validation is based on published data (PMID: 31651360).
同种型:
IgG2a
免疫原:
Recombinant protein encompassing a sequence within the N-terminus region of human C9orf72. The exact sequence is proprietary.
克隆性:
Monoclonal
克隆号:
GT1553
纯化方式:
Affinity purified by Protein A.
偶联:
Unconjugated
产品浓度:
1 mg/ml (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
dry ice
产品形式:
Liquid
存储溶液:
PBS, no preservatives.
生产商:
GeneTex
功能与背景:
The protein encoded by this gene plays an important role in the regulation of endosomal trafficking, and has been shown to interact with Rab proteins that are involved in autophagy and endocytic transport. Expansion of a GGGGCC repeat from 2-22 copies to 700-1600 copies in the intronic sequence between alternate 5' exons in transcripts from this gene is associated with 9p-linked ALS (amyotrophic lateral sclerosis) and FTD (frontotemporal dementia) (PMID: 21944778, 21944779). Studies suggest that hexanucleotide expansions could result in the selective stabilization of repeat-containing pre-mRNA, and the accumulation of insoluble dipeptide repeat protein aggregates that could be pathogenic in FTD-ALS patients (PMID: 23393093). Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2016]
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