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Merck
CN

MAB2160

脆性X智力低下蛋白抗体，克隆1C3

ascites fluid, clone 1C3, Chemicon®

别名:

FMRP

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关于此项目

UNSPSC Code:
12352203
NACRES:
NA.41
eCl@ss:
32160702

主要文件

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产品名称

脆性X智力低下蛋白抗体，克隆1C3, ascites fluid, clone 1C3, Chemicon®

biological source

mouse

conjugate

unconjugated

antibody form

ascites fluid

antibody product type

primary antibodies

clone

1C3, monoclonal

species reactivity

mouse, human, rat

manufacturer/tradename

Chemicon®

technique(s)

ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
western blot: suitable

isotype

IgG1κ

suitability

not suitable for flow cytometry
not suitable for immunoprecipitation

NCBI accession no.

NM_002024.3

UniProt accession no.

Q06787

shipped in

dry ice

target post-translational modification

unmodified

Quality Level

100

Gene Information

human ... FRAXA(2477), FRAXA(108684022)

相关类别

Application

脆性X智力低下蛋白抗体（克隆1C3）可检测脆性X智力低下蛋白水平 & 已发表 & 经验证可用于ELISA、IC、IH(P) & WB。
免疫组化：
冷冻和石蜡切片。使用先前批次的1:500-1:5,000稀释液。

ELISA：
将先前批次的1:500-1:5,000稀释液用于ELISA。

血液涂片上FMRP的检测：
使用先前批次的1:500-1:5,000稀释液。

免疫细胞化学：
将先前批次的1:500-1:5,000稀释液用于转染细胞。轻度固定（2% PFA，仅在封闭过程中用0.1% triton通透）

最佳工作稀释度必须由最终用户进行确定。
研究子类别
神经退行性疾病

RNA结合蛋白（RBP）
研究类别
神经科学

Analysis Note

对照
HeLa全细胞裂解液、HeLa核裂解液、小鼠E17脊髓裂解液
通过蛋白质印迹法对小鼠E17脊髓裂解液进行了评估。

蛋白质印迹分析：
该抗体的1:500稀释液在10 μg小鼠E17脊髓裂解液中检测到脆性X智力低下蛋白。

Biochem/physiol Actions

人和小鼠FMRP。其表位位于FMRP的N端半部分。在FXR蛋白高表达的情况下，可检测到其与FXR蛋白交叉反应。

Disclaimer

除非我们的产品目录或产品附带的其他公司文档另有说明，否则我们的产品仅供研究使用，不得用于任何其他目的，包括但不限于未经授权的商业用途、体外诊断用途、离体或体内治疗用途或任何类型的消费或应用于人类或动物。

General description

~71 kDa
脆性X智力低下蛋白（FMRP）是一种与多核糖体相关的RNA结合蛋白，可能参与mRNA从细胞核到细胞质的转运。FMR1缺陷是脆性X综合征的病因，这是一种常见的遗传性疾病，其特征是中度至重度智力低下、巨睾症、大耳朵、下颌突出以及言语高亢、诙谐。大多数脆性X综合征患者的缺陷是由编码区正前方的CGG重复区扩增引起的。

Immunogen

具有全长FMRP（人）的融合蛋白。

Other Notes

浓度：请参考批次特异性浓缩物的检验报告。

Physical form

未纯化
腹水小鼠单克隆IgG1k液体，不含防腐剂

Preparation Note

自收到之日起在-20ºC可稳定保存1年。

Legal Information

CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany

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存储类别

10 - Combustible liquids

wgk

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

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The RNA-binding protein fragile X-related 1 regulates somite formation in Xenopus laevis.
Huot, ME; Bisson, N; Davidovic, L; Mazroui, R; Labelle, Y; Moss, T; Khandjian, EW
Molecular Biology of the Cell null
Gabi Schutzius et al.
Journal of neurodevelopmental disorders, 5(1), 8-8 (2013-04-04)
Hypermethylation of the fragile X mental retardation 1 gene FMR1 results in decreased expression of FMR1 protein FMRP, which is the underlying cause of Fragile X syndrome - an incurable neurological disorder characterized by mental retardation, anxiety, epileptic episodes and
FMRP S499 is phosphorylated independent of mTORC1-S6K1 activity.
Bartley, CM; O'Keefe, RA; Bordey, A
Testing null
Alice Filippini et al.
RNA biology, 14(11), 1580-1591 (2017-06-24)
The fragile X syndrome (FXS), the most common form of inherited intellectual disability, is due to the absence of FMRP, a protein regulating RNA metabolism. Recently, an unexpected function of FMRP in modulating the activity of Adenosine Deaminase Acting on
Julie B Zang et al.
PLoS genetics, 5(12), e1000758-e1000758 (2009-12-17)
The mental retardation, autistic features, and behavioral abnormalities characteristic of the Fragile X mental retardation syndrome result from the loss of function of the RNA-binding protein FMRP. The disease is usually caused by a triplet repeat expansion in the 5'UTR
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相关内容

RNA-Binding Protein Immunoprecipitation

All eukaryotic organisms require tight regulation of gene expression for complex processes such as development, differentiation, cell specification, and responses to environmental stimuli. Many genes are regulated post-transcriptionally, in addition to transcriptional mechanisms of gene regulation. RNA-binding proteins (RBPs) are essential for post-transcriptional gene regulation, linking transcription and translation in many processes including transcription, splicing, export, rate of translation and turnover. In all of these events, RBPs coordinate regulation of the amount of protein produced from mRNA transcripts.

Anti-Fragile X Mental Retardation Protein, clone 1C3 - Data Sheet

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