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.

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.

Mouse,Human,Rat,Dog,Cow,Rabbit,Sheep

WB

WB 1:500-2000

52kD

10658

Q92879

https://www.uniprot.org/uniprotkb/Q92879

Nucleus. Cytoplasm. RNA-binding activity is detected in both nuclear and cytoplasmic compartments.

Ubiquitous.

KLH conjugated synthetic peptide derived from human CUG-BP1

Rabbit

Polyclonal

IgG

affinity purified by Protein A

Liquid

Preservative: 0.02% Proclin300, Constituents: 1% BSA, 0.01M PBS, pH7.4.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.