Thermo Fisher Scientific CUGBP1 Polyclonal Antibody

Thermo Fisher Scientific CUGBP1 Polyclonal Antibody

Applications and Tested Dilution

Product Specifications

Product Specific Information

Immunogen sequence:
KRMAQQLQQQ MQQISAASVW GNLAGLNTLG PQYLALYLQL LQQTASSGNL NTLSSLHPMG GLNAMQLQNL AA

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 isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50.
The 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.

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