Thermo Fisher Scientific CUGBP1 Recombinant Rabbit Monoclonal Antibody (ARC1866)

Applications and Tested Dilutions

Product Specifications

Product Specific Information

• Positive Samples: SH-SY5Y, Mouse brain, Rat brain
• Immunogen sequence:
  MNGTLDHPDQ PDLDAIKMFV GQVPRTWSEK DLRELFEQYG AVYEINVLRD RSQNPPQSKG CCFVTFYTRK AALEAQNALH NMKVLPGMHH PIQMKPADSE

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, CUG-BP1, plays 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 the heterogeneous nuclear ribonucleoprotein (hnRNP) hNab50.
CUG-BP/hNab50 is localized predominantly in the nucleus and associates with polyadenylated RNAs in vivo. RNA-binding and 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 serves as a binding site for CUG-BP/hNab50 in vivo, and 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.