PQBP1抗体PQBP1兔多抗抗体Sutherland Ha
an X linked mental retardation syndrome antibody抗体PQBP1 Antibody抗体- ￥440 - 1320
- CUSABIO
- CN
- CSB-PA018594LA01HU
- 2025年07月14日
- ELISA, IHC; Recommended dilution: IHC:1:20-1:200
- Rabbit
- Human
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- 详细信息
- 文献和实验
- 技术资料
- 免疫原：
Recombinant Human Polyglutamine-binding protein 1 protein (2-265AA)
- 亚型：
IgG
- 形态：
Liquid
- 保存条件：
Upon receipt, store at -20℃ or -80℃. Avoid repeated freeze.
- 克隆性：
Polyclonal
- 标记物：
Non-conjugated
- 适应物种：
Human
- 保质期：
6个月
- 抗原来源：
Homo sapiens (Human)
- 目录编号：
O60828
- 级别：
优
- 库存：
200
- 供应商：
武汉华美生物工程有限公司
- 宿主：
Rabbit
- 应用范围：
ELISA, IHC; Recommended dilution: IHC:1:20-1:200
- 浓度：
>95%,Protein G purified
- 靶点：
PQBP1
- 抗体英文名：
PQBP1 Antibody
- 抗体名：
Sutherland Haan X linked mental retardation syndrome antibody
- 规格：
100μg/50μg/20μg
| 规格： | 100μg | 产品价格： | ￥1320.0 |
|---|---|---|---|
| 规格： | 50μg | 产品价格： | ￥880.0 |
| 规格： | 20μg | 产品价格： | ￥440.0 |
保存缓冲液
Preservative: 0.03% Proclin 300Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
功能
Probably functions as scaffold protein that is part of numerous complexes and thereby plays a role in pre-mRNA splicing, transcription regulation and neuron development. Required for normal alternative splicing of target pre-mRNA species (PubMed:23512658). May suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. Can activate transcription directly or via association with the transcription machinery. May be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit.风险提示：丁香通仅作为第三方平台，为商家信息发布提供平台空间。用户咨询产品时请注意保护个人信息及财产安全，合理判断，谨慎选购商品，商家和用户对交易行为负责。对于医疗器械类产品，请先查证核实企业经营资质和医疗器械产品注册证情况。
文献和实验Identification of Messenger RNAs and MicroRNAs Associated With Fragile X Mental Retardation Protein
Fragile X syndrome, a common form of inherited mental retardation, is caused by the loss of the Fragile X mental retardation protein (FMRP). FMRP, which may regulate translation in neurons, not only associates with specific messenger RNAs
Mutation Analysis of X-Linked Emery-Dreifuss Muscular Dystrophy Gene
. For this reason, the term “Emery-Dreifuss syndrome” was proposed for the triad of symptoms (6 ) Molecular diagnosis is therefore very important for an early diagnosis, and to distinguish X-linked EDMD from the autosomal forms, as well as from different MDs
Microdeletion Syndromes: Characteristics and Diagnosis
X-Linked Ichthyosis, Mental Retardation, Epilepsy, Ocular Albinism, Kallman Syndrome Xp22.3 Investigator Xp21 Deletion
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