Thermo Fisher Scientific CUGBP1 Monoclonal Antibody (3B1)

Applications and Tested Dilutions

Product Specifications

Product Specific Information

Suggested positive control: antigen standard for CUGBP1 (transient overexpression lysate).

Target Information

Myotonic dystrophy (MD) is an autosomal dominant neuromuscular disease associated with a (CTG)n repeat expansion in the 3′-untranslated region of the myotonin protein kinase (Mt-PK) gene. A (CUG)n oligonucleotide triplet repeat pre-mRNA/mRNA binding protein may play an important role in DM pathogenesis.
HeLa cell protein, CUG-BP1, has been purified based on its ability to bind specifically to (CUG)8 oligonucleotides in vitro. CUG-BP1 is the major (CUG)8 binding activity in normal cells and has been identified as an isoform of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50.
CUG-BP/hNab50 protein is localized predominantly 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.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.