- 当前位置：首页 > >Merck Millipore > 生命科学研究
- 生命科学研究
-
-
merck millipore,默克密理博,MAB2160,Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3
产品名称：Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3
产品型号：MAB2160
Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3 detects level of Fragile X Mental Retardation Protein & has been published & validated for use in ELISA, IC, IH, IH(P) & WB.
merck millipore,默克密理博,MAB2160,Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 R, H, M WB, ELISA, ICC, IHC M Ascites Monoclonal Antibody 描述 产品目录编号 MAB2160 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3 Alternate Names - FMRP
背景信息 Fragile X Mental Retardation Protein (FMRP) is a RNA-binding protein that is associated to polysomes and may be involved in the transport of mRNA from the nucleus to the cytoplasm. Defects in FMR1 are the cause of Fragile X syndrome, which is a common genetic disease characterized by moderate to severe mental retardation, macroorchidism, large ears, prominent jaw, and high-pitched, jocular speech. The defect in most fragile X syndrome patients results from an amplification of a CGG repeat region which is directly in front of the coding region. 产品信息 格式 Ascites 控制 - HeLa whole cell lysate, HeLa nuclear lysate, mouse E17 spinal cord lysate
演示 Ascites mouse monoclonal IgG1k fluid containing no preservatives 应用 应用 Anti-Fragile X Mental Retardation Protein Antibody, clone 1C3 detects level of Fragile X Mental Retardation Protein & has been published & validated for use in ELISA, IC, IH, IH(P) & WB. 主要应用 - Western Blotting
- ELISA
- Immunocytochemistry
- Immunohistochemistry
不建议的应用 - Flow Cytometry
应用说明 Immunohistochemistry:
Frozen and paraffin sections. 1:500-1:5,000 dilution of a previous lot was used.
ELISA:
A 1:500-1:5,000 dilution of a previous lot was used in ELISA.
Detection of FMRP on Blood Smears:
A 1:500-1:5,000 dilution of a previous lot was used.
Immunocytochemistry:
A 1:500-1:5,000 dilution of a previous lot was used on transfected cells. Light fixation (2% PFA, permeabilize with 0.1% triton in block only)
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Fusion protein with a full length FMRP (human). 克隆 1C3 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 Human and mouse FMRP. The epitope is localized in the N-terminal half of FMRP. Cross reaction with FXR protein may be detected in cases of high expression of the latter proteins. 同种型 IgG1κ 品种反应性 RatHumanMouse Species Reactivity Note Human, mouse and rat. 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_002024.3
Entrez基因汇总 The protein encoded by this gene binds RNA and is associated with polysomes. The encoded protein may be involved in mRNA trafficking from the nucleus to the cytoplasm. A trinucleotide repeat (CGG) in the 5' UTR is normally found at 6-53 copies, but an expansion to 55-230 repeats is the cause of fragile X syndrome. 基因符号 - MGC87458
- FMR1
- FRAXA
- FMRP
纯化方法 Unpurified UniProt编号 - Q06787
UniProt汇总 FUNCTION: SwissProt: Q06787 # RNA-binding protein. Associated with polysomes and might be involved in the transport of mRNA from the nucleus to the cytoplasm. Binds strongly to poly(G), binds moderately to poly(U) but shows very little binding to poly(A) or poly(C).
SIZE: 632 amino acids; 71174 Da
SUBUNIT: Found in a RNP granule complex with IGF2BP1. Interacts with FXR1, FXR2, IGF2BP1, NUFIP1 and RANBP9. Interacts with CYFIP1 and CYFIP2.
SUBCELLULAR LOCATION: Cytoplasm. Nucleus.
TISSUE SPECIFICITY: Highest levels found in neurons, brain, testis, placenta and lymphocytes. Also expressed in epithelial tissues and at very low levels in glial cells.
DISEASE: SwissProt: Q06787 # Defects in FMR1 are the cause of fragile X syndrome. [MIM:300624]. It is a common genetic disease (has a prevalence of one in every 2000 children) which is characterized by moderate to severe mental retardation, macroorchidism (enlargement of the testicles), large ears, prominent jaw, and high-pitched, jocular speech. The defect in most fragile X syndrome patients results from an amplification of a CGG repeat region which is directly in front of the coding region. & Defects in FMR1 are the cause of fragile X tremor/ataxia syndrome (FXTAS) [MIM:300623]. In FXTAS, the expanded repeats range in size from 55 to 200 repeats and are referred to as 'premutations'. Full repeat expansions with greater than 200 repeats results in fragile X mental retardation syndrome [MIM:300624]. Carriers of the premutation typically do not show the full fragile X syndrome phenotype, but comprise a subgroup that may have some physical features of fragile X syndrome or mild cognitive and emotional problems.
SIMILARITY: SwissProt: Q06787 ## Belongs to the FMR1 family. & Contains 2 KH domains.
MISCELLANEOUS: RNA-binding activity is inhibited by RANBP9. & The mechanism of the severe phenotype in the Asn- 304 patient lies in the sequestration of bound mRNAs in nontranslatable mRNP particles. In the absence of FMRP, these same mRNAs may be partially translated via alternate mRNPs, although perhaps abnormally localized or regulated, resulting in typical fragile X syndrome. Asn-304 mutation maps to a position within the second KH domain of FMRP that is critical for stabilizing sequence-specific RNA-protein interactions. Asn-304 mutation abrogates the association of the FMRP KH 2 domain with its target, kissing complex RNA.产品使用声明 质量保证 Evaluated by Western Blot on Mouse E17 spinal cord lysates.
Western Blotting Analysis:
1:500 dilution of this antibody detected Fragile X Mental Retardation Protein on 10 μg of Mouse E17 spinal cord lysates.使用声明 - 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.
储存和货运信息 存储条件 Stable for 1 year at -20ºC from date of receipt. 包装信息 数量 100 µL
上一件merck millipore产品：merck millipore,默克密理博,MAB2013,Anti-K-Cadherin Antibody, extracellular domain, clone 2B6
下一件merck millipore产品：merck millipore,默克密理博,SF-1074,FBXW7; Human, Cyanine 3, RNA Detection Probe,SmartFlare
相关产品
merck millipore,默克密理博,MABF193,Anti-CD32 Antibody, clone EP6243, rabbit monoclonal
merck millipore,默克密理博,07-1387,Anti-oxidized-CaM Kinase II (Met281/282) Antibody
merck millipore,默克密理博,FCMAB221PE,Milli-Mark™ Anti-Interleukin-2-PE Antibody, Clone S4B6-1
merck millipore,默克密理博,MABE331,Anti-MDM2 Antibody, clone 4B2C1.11
merck millipore,默克密理博,MAB2013,Anti-K-Cadherin Antibody, extracellular domain, clone 2B6
merck millipore,默克密理博,SF-1074,FBXW7; Human, Cyanine 3, RNA Detection Probe,SmartFlare
merck millipore,默克密理博,MABF354,Anti-CD2 (mouse) Antibody, PE, clone RM2-5
