missing translation for 'onlineSavingsMsg'
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Description
Myotonic dystrophy (MD) is an autosomal domit neuromuscular disease that is associated with a (CTG)n repeat expansion in the 3′-untranslated region of the myotonin protein kinase (Mt-PK) gene. A (CUG) n oligonucleotides triplet repeat pre-mRNA/mRNA binding protein may play an important role in DM pathogenesis. HeLa cell protein, CUG-BP1, has been purified based upon its ability to bind specifically to (CUG) 8 oligonucleotides in vitro. CUG-BP1 is the major (CUG) 8 binding activity in normal cells. CUG-BP1 has been identified as isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50. The CUG-BP/hNab50 protein is localized predomitly in the nucleus and is associated with polyadenylated RNAs in vivo. In vitro RNA-binding/photocrosslinking studies demonstrate that CUG-BP/hNab50 binds to RNAs containing the Mt-PK 3-UTR. The (CUG) n repeat region in Mt-PK mRNA is a binding site for CUG-BP/hNab50 in vivo, and triplet repeat expansion leads to sequestration of this hnRNP on mutant Mt-PK transcripts.
Specifications
Specifications
| Antigen | CUGBP1 |
| Applications | Immunofluorescence, Western Blot, Immunohistochemistry (Paraffin), Immunoprecipitation, Immunocytochemistry |
| Classification | Polyclonal |
| Concentration | 0.37 mg/mL |
| Conjugate | Unconjugated |
| Formulation | PBS with 50% glycerol and 0.02% sodium azide; pH 7.3 |
| Gene | CELF1 |
| Gene Accession No. | P28659, Q4QQT3, Q92879 |
| Gene Alias | Bruno like protein 2, BRUNOL2, CELF 1, CELF1, CUG BP, CUG BP1, CUGBP, CUGBP1, Deadenylation factor CUG BP, EDEN BP homolog, hNab50, NAB50, RNA binding protein BRUNOL 2 |
| Gene Symbols | Celf1 |
| Show More |
Product Title
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