Thermo Fisher Scientific CUGBP1 Monoclonal Antibody (OTI5B8)

Applications and Tested Dilution

Product Specifications

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, binds specifically to (CUG)₈ oligonucleotides in vitro and represents 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 predominantly nuclear and associates with polyadenylated RNAs in vivo. RNA-binding studies indicate that CUG-BP/hNab50 interacts with RNAs containing the Mt-PK 3′-UTR, 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.

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