| 克隆类型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from human CUG-BP1 (442-486aa). |
|
纯化方式 ： |
Immunogen affinity purified |
|
缓 冲 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
产品描述 ： |
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. |
| 适用物种 ： |
Hu, Mo, Rat, Chi, Dog, Pig, Cow, Hor, Sheep |
| 应用说明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 别 名 ： |
Bruno like 2; bruno like protein 2; BRUNOL2; 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-BP; CUG-BP- and ETR-3-like factor 1; CUGBP 1; CUGBP and ETR3 like factor 1; CUGBP; 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 homolog; EDEN-BP; EDEN-BP homolog; embryo deadenylation element binding protein; embryo deadenylation element binding protein homolog; Embryo deadenylation element-binding protein homolog; hNab50; NAB50; NAPOR; Nuclear polyadenylated RNA binding protein 50 kD; Nuclear polyadenylated RNA binding protein; RNA binding protein BRUNOL2; RNA-binding protein BRUNOL-2; 50 kDa Nuclear polyadenylated RNA binding protein. |
| 保存条件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 标 记 物 ： |
PLLABS公司可供应生物素、辣根过氧化物酶、荧光素标记抗体 |
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产品说明 ： |
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