磷酸化脆性X综合征相关蛋白AFF1抗体

英文名称    phospho-FMRP (Ser500)    

中文名称    磷酸化脆性X综合征相关蛋白AFF1抗体    

别    名    FMRP (phospho S500); FMRP (phospho Ser500); p-FMRP (phospho S500); p-FMR1 (phospho S500); FMR1; FMR1_HUMAN; FMRP; FMRP phospho S499; Fragile X mental retardation 1; Fragile X mental retardation 1 protein; Fragile X mental retardation protein 1; FRAXA; MGC87458; POF; POF1; Protein FMR-1; Protein FMR1.    

说 明 书    0.1ml      

产品类型    磷酸化抗体     

研究领域    细胞生物  神经生物学  信号转导  细胞类型标志物  表观遗传学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Zebrafish, Sheep, Guinea Pig, Danio rerio    

产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 磷酸化脆性X综合征相关蛋白AFF1抗体（石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    75kDa    

细胞定位    细胞核 细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthesised phosphopeptide derived from human FMRP around the phosphorylation site of Ser500    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存条件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

磷酸化脆性X综合征相关蛋白AFF1抗体产品介绍    background:

?Fragile X syndrome is the most frequent form of inherited mental retardation and is the result of transcriptional silencing of the FMR1 gene on the X chromosome. The FMR1 gene contains a distinct CpG dinucleotide repeat located in the 5' untranslated region of the gene. In fragile X syndrome this tandem repeat is substantially amplified and subjected to extensive methylation and enhanced transcriptional silencing. The FMR1 protein (or FMRP) is an RNA-binding protein that associates with polyribosomes and is a likely component of a messenger ribonuclear protein (mRNP) particle. It contains several features that are characteristics of RNA-binding proteins, including two hnRNPK homology (KH) domains and an RGG amino acid motif (RGG box). FMR1 localizes to both the nucleus and the cytoplasm and can also interact with two fragile X syndrome related factors, FXR1 and FXR2, which form heterodimers through their N-terminal coiled-coil domains. Since FMR1 contains both a nuclear localization signal and a nuclear export signal it is also implicated in the nucleocytoplasmic transport of mRNAs.

Function:
Translation repressor. Component of the CYFIP1-EIF4E-FMR1 complex which binds to the mRNA cap and mediates translational repression. In the CYFIP1-EIF4E-FMR1 complex this subunit mediates translation repression (By similarity). RNA-binding protein that plays a role in intracellular RNA transport and in the regulation of translation of target mRNAs. Associated with polysomes. May play a role 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).

Subunit:
Component of the CYFIP1-EIF4E-FMR1 complex which is composed of CYFIP, EIF4E and FMR1. Interacts with CYFIP1 and CYFIP2. The interaction with brain cytoplasmic RNA 1 (BC1) increases binding affinity for the CYFIP1-EIF4E complex in the brain (By similarity). Homooligomer. Found in a RNP granule complex with IGF2BP1. Directly interacts with SMN and TDRD3. Interacts with the SMN core complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts with FXR1, FXR2, IGF2BP1, NUFIP1, NUFIP2, MCRS1 and RANBP9.

Subcellular Location:
Cytoplasm. Nucleus, nucleolus.

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.

Post-translational modifications:
Phosphorylated on several serine residues.

DISEASE:
Defects in FMR1 are the cause of fragile X syndrome (FRAX) [MIM:300624]. Fragile X syndrome 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. 
Defects in FMR1 are the cause of premature ovarian failure syndrome type 1 (POF1) [MIM:311360]. An ovarian disorder defined as the cessation of ovarian function under the age of 40 years. It is characterized by oligomenorrhea or amenorrhea, in the presence of elevated levels of serum gonadotropins and low estradiol.

Similarity:
Belongs to the FMR1 family.
Contains 2 Agenet-like domains.
Contains 2 KH domains.

Database links:
UniProtKB/Swiss-Prot: Q06787.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.    

磷酸化脆性X综合征相关蛋白AFF1抗体

hz-0293M-PE-hzy7 Mouse Anti-rat IgG/PE-Cy7  PE-Cy7标记的小鼠抗大鼠IgG

hz-0326M-Bio Mouse Anti-Bov IgG/Bio  生物素标记的小鼠抗牛IgG

hz-0326M-hzy3 Mouse Anti-Bov IgG/Cy3  Cy3标记的小鼠抗牛IgG

hz-0326M-AP Mouse Anti-Bov IgG/AP  碱性磷酸酶（AP）标记的小鼠抗牛IgG

hz-0326M-PE-hzy7 Mouse Anti-Bov IgG/PE-Cy7  PE-Cy7标记的小鼠抗牛IgG

hz-0326M-Gold Mouse Anti-Bov IgG/Gold  胶体金标记的小鼠抗牛IgG

hz-0326M-HRP Mouse Anti-Bov IgG/HRP  辣根过氧化物酶标记的小鼠抗牛IgG

hz-0326M-RBIThz Mouse Anti-Bov IgG/RBITC  罗丹明标记的小鼠抗牛IgG

hz-0326M-FIThz Mouse Anti-Bov IgG/FITC  FITC标记的小鼠抗牛IgG

hz-0326M-hzy5 Mouse Anti-Bov IgG/Cy5  Cy5标记的小鼠抗牛IgG

hz-0326M-hzy5.5 Mouse Anti-Bov IgG/Cy5.5  Cy5.5标记的小鼠抗牛IgG

hz-0326M-hzy7 Mouse Anti-Bov IgG/Cy7  Cy7标记的小鼠抗牛IgG

hz-0326M-PE Mouse Anti-Bov IgG/PE  PE标记的小鼠抗牛IgG

hz-0326M-PE-hzy3 Mouse Anti-Bov IgG/PE-Cy3  PE-Cy3标记的小鼠抗牛IgG

hz-0326M-PE-hzY5 Mouse Anti-Bov IgG/PE-CY5  PE-CY5标记的小鼠抗牛IgG

hz-0326M-APhz Mouse Anti-Bov IgG/APC  APC标记的小鼠抗牛IgG

hz-0326M-AF350 Mouse Anti-Bov IgG/Alexa Fluor 350  Alexa Fluor 350标记的小鼠抗牛IgG

hz-0326M-AF488 Mouse Anti-Bov IgG/Alexa Fluor 488  Alexa Fluor 488标记的小鼠抗牛IgG

hz-0326M-AF555 Mouse Anti-Bov IgG/Alexa Fluor 555  Alexa Fluor 555标记的小鼠抗牛IgG

hz-0326M-AF647 Mouse Anti-Bov IgG/Alexa Fluor 647  Alexa Fluor 647标记的小鼠抗牛IgG

hz-0326M-PE-hzy5.5 Mouse Anti-Bov IgG/PE-Cy5.5  PE-Cy5.5标记的小鼠抗牛IgG

hz-0358M-hzy3 Mouse Anti-Guinea pig IgG/Cy3  Cy3标记的小鼠抗豚鼠IgG

hz-0358M-hzy5 Mouse Anti-Guinea pig IgG/Cy5  Cy5标记的小鼠抗豚鼠IgG

hz-0358M-hzy5.5 Mouse Anti-Guinea pig IgG/Cy5.5  Cy5.5标记的小鼠抗豚鼠IgG

hz-0358M-hzy7 Mouse Anti-Guinea pig IgG/Cy7  Cy7标记的小鼠抗豚鼠IgG

hz-0358M-PE Mouse Anti-Guinea pig IgG/PE  PE标记的小鼠抗豚鼠IgG

hz-0358M-PE-hzy3 Mouse Anti-Guinea pig IgG/PE-Cy3  PE-Cy3标记的小鼠抗豚鼠IgG

hz-0358M-PE-hzY5 Mouse Anti-Guinea pig IgG/PE-CY5  PE-CY5标记的小鼠抗豚鼠IgG

hz-0358M-FIThz Mouse Anti-Guinea pig IgG/FITC  FITC标记的小鼠抗豚鼠IgG

hz-0358M-RBIThz Mouse Anti-Guinea pig IgG/RBITC  罗丹明标记的小鼠抗豚鼠IgG

hz-0358M-AP Mouse Anti-Guinea pig IgG/AP  碱性磷酸酶（AP）标记的小鼠抗豚鼠IgG

hz-0358M-Bio Mouse Anti-Guinea pig IgG/Bio  生物素标记的小鼠抗豚鼠IgG