Myotonic dystrophy (MD) is an autosomal dominant 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 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.
Applications:Suitable for use in Western Blot, Immunoprecipitation, Immunocytochemistry and Supershift Assay.
Other applications have not been tested.
Recommended Dilutions:Western Blot: 0.1-2.0ug/ml detects CUG-BP1 in HeLa nuclear extract.
HeLa nuclear extract was resolved by electrophoresis, transferred to nitrocellulose and probed with C7948-50 (0.5ug/ml).
Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and a chemiluminescence detection system.
Immunoprecipitation: Immunoprecipitates CUG-BP1 from HeLa cell lysates
Immunocytochemistry: Nuclear staining in HeLa and myoblasts from normal patients.
Supershift: Supershifts CUG-BP1:DNA complexes in EMSA
Optimal dilutions to be determined by the researcher.