Rabbit Anti-CUG-BP1/FITC Conjugated antibody
|别 名||Bruno like 2; bruno like protein 2; Bruno-like protein 2; BRUNOL 2; BRUNOL2; CELF 1; CELF-1; celf1; CELF1 CUGBP, Elav like family member 1; CELF1_HUMAN; CUG BP and ETR 3 like factor 1; CUG BP; CUG BP1; CUG RNA binding protein; CUG triplet repeat RNA binding protein 1; CUG triplet repeat RNA-binding protein 1; CUG-BP; CUG-BP- and ETR-3-like factor 1; CUG-BP1; CUGBP 1; CUGBP and ETR3 like factor 1; CUGBP; CUGBP Elav like family member 1; CUGBP Elav-like family member 1; CUGBP1; Cytidine uridine guanosine binding protein 1; Deadenylation factor CUG BP; Deadenylation factor CUG-BP; Deadenylation factor CUGBP; EDEN BP; EDEN BP homolog; EDEN-BP; EDEN-BP homolog; embryo deadenylation element binding protein; embryo deadenylation element binding protein homolog; Embryo deadenylation element-binding protein homolog; hNab 50; hNab50; NAB 50; NAB50; NAPOR; Nuclear polyadenylated RNA binding protein 50 kD; Nuclear polyadenylated RNA binding protein; RNA binding protein BRUNOL 2; RNA binding protein BRUNOL2; RNA-binding protein BRUNOL-2; 50 kDa Nuclear polyadenylated RNA binding protein; 50 kDa nuclear polyadenylated RNA-binding protein.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
|研究领域||细胞生物 发育生物学 染色质和核信号 转录调节因子 表观遗传学|
|交叉反应||Human, Mouse, Rat, Dog, Cow, Rabbit, Sheep,|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||52kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human CUG-BP1|
|纯化方法||affinity purified by Protein A|
|储 存 液||0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.|
|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.|
Myotonic dystrophy (DM) is an autosomal dominant neuromuscular disease that is associated with a (CTG)n repeat expansion in the 3’-untranslated region of the myotonin protein kinase gene (DMPK). CUG-BP1 and CUG-BP2 are proteins that bind specifically to (CUG)8 oligonucleotides in vitro. While CUG-BP1 has the major binding activity in normal cells, nuclear CUG-BP2 binding activity increases in DM cells. Both CUG-BP1 and CUG-BP2 are isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50. CUG-BP1, an RNA CUG triplet repeat binding protein, regulates splicing and translation of various RNAs. Expansion of RNA CUG repeats in the DMPK in DM is associated with alterations in binding activity of CUG-BP1 as well as alterations in the translation of the C/EBPb transcription factor. CUG-BP1 is an important regulator of initiation from different AUG codons of C/EBPb mRNA. In normal cells, CUG-BP1 up-regulates the p21 protein during differentiation by inducing the translation of p21 via binding to a GC-rich sequence located within the 5’ region of p21 mRNA. In DM cells, failure to accumulate CUG-BP1 leads to a reduction of p21 and alterations in other proteins responsible for cell cycle withdrawl.
RNA-binding protein implicated in the regulation of several post-transcriptional events. Involved in pre-mRNA alternative splicing, mRNA translation and stability. Mediates exon inclusion and/or exclusion in pre-mRNA that are subject to tissue-specific and developmentally regulated alternative splicing. Specifically activates exon 5 inclusion of cardiac isoforms of TNNT2 during heart remodeling at the juvenile to adult transition. Acts as both an activator and repressor of a pair of coregulated exons: promotes inclusion of the smooth muscle (SM) exon but exclusion of the non-muscle (NM) exon in actinin pre-mRNAs. Activates SM exon 5 inclusion by antagonizing the repressive effect of PTB. Promotes exclusion of exon 11 of the INSR pre-mRNA. Inhibits, together with HNRNPH1, insulin receptor (IR) pre-mRNA exon 11 inclusion in myoblast. Increases translation and controls the choice of translation initiation codon of CEBPB mRNA. Increases mRNA translation of CEBPB in aging liver (By similarity). Increases translation of CDKN1A mRNA by antagonizing the repressive effect of CALR3. Mediates rapid cytoplasmic mRNA deadenylation. Recruits the deadenylase PARN to the poly(A) tail of EDEN-containing mRNAs to promote their deadenylation. Required for completion of spermatogenesis (By similarity). Binds to (CUG)n triplet repeats in the 3'-UTR of transcripts such as DMPK and to Bruno response elements (BREs). Binds to muscle-specific splicing enhancer (MSE) intronic sites flanking the alternative exon 5 of TNNT2 pre-mRNA. Binds to AU-rich sequences (AREs or EDEN-like) localized in the 3'-UTR of JUN and FOS mRNAs. Binds to the IR RNA. Binds to the 5'-region of CDKN1A and CEBPB mRNAs. Binds with the 5'-region of CEBPB mRNA in aging liver.
Component of an EIF2 complex at least composed of CELF1/CUGBP1, CALR, CALR3, EIF2S1, EIF2S2, HSP90B1 and HSPA5. Associates with polysomes (By similarity). Interacts with HNRNPH1; the interaction in RNA-dependent. Interacts with PARN.
Nucleus. Cytoplasm. RNA-binding activity is detected in both nuclear and cytoplasmic compartments.
Phosphorylated. Its phosphorylation status increases in senescent cells.
Belongs to the CELF/BRUNOL family.
Contains 3 RRM (RNA recognition motif) domains.
Entrez Gene: 10658 Human
Entrez Gene: 13046 Mouse
Entrez Gene: 362160 Rat
Omim: 601074 Human
SwissProt: Q92879 Human
SwissProt: P28659 Mouse
SwissProt: Q4QQT3 Rat
Unigene: 595333 Human
Unigene: 29495 Mouse
Unigene: 393354 Mouse
Unigene: 22432 Rat
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
肌强直性营养不良（DM）是一种常染色体显性神经肌肉疾病，与肌强直蛋白激酶基因（DMPK）3’非翻译区CTG n重复扩增有关。CuG-BP1和CuG-BP2是与（CuG）8寡核苷酸特异结合的蛋白。CUG-BP1在正常细胞中主要具有结合活性，而在DM细胞中核CUG-BP2结合活性增加。CUG-BP1和CUG-BP2都是新的异质核糖核蛋白(hnRNP)hNab50的同型体。CUG-BP1是一种RNA CUG三重重复结合蛋白，调节各种RNA的剪接和翻译。DM中DMPK中RNA CUG重复序列的扩展与CUG-BP1结合活性的改变以及C/EBPb转录因子翻译的改变有关。CuG-BP1是C／EBPB mRNA不同Ag密码子启动的重要调控因子。在正常细胞中，CUG-BP1通过与位于p21 mRNA 5′区的富含GC的序列结合来诱导p21的翻译，从而在分化过程中上调p21蛋白。在DM细胞中，CUG-BP1积累失败导致p21的减少和其他导致细胞周期撤退的蛋白质的改变。