CUG-Binding Protein 1 Ribonucleoprotein (CUG-BPI, Cytidine Uridine Guanosine Binding Protein 1)

Antibodies / Antibodies-Nuclear Proteins

-20°C

Supplied as a liquid in Tris-Glycine, 0.15M sodium chloride, 0.02% sodium azide.

CUG-BP1 human nuclear RNA binding protein.

Myotonic dystrophy (MD) is an autosomal dominant neuromuscular disease that is associated with a (CTG)n repeat expansion in the 3í-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 n 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 3í-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.

Applications:
Suitable for use in Flow Cytometry, Western Blot, Immunohistochemistry and Immunofluorescence/Immunocytochemistry. Other applications have not been tested.

Recommended Dilutions:
Western Blot: 1:1000, detects a ~50kD band
Immunoprecipitation: 1:10-1:500
Immunohistochemistry (Frozen): 1:10-1:500
Immunohistochemistry (Paraffin): 1:10-1:500
Immunofluorescence (IC): 1:10-1:500
Flow Cytometry: 1ug/10e6 cells
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.