Technical Data
C7948-50A
CUG-Binding Protein 1 Ribonucleoprotein (CUG-BPI, Cytidine Uridine Guanosine Binding Protein 1)
Description:
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 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 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.

Applications:
Suitable for use in ELISA. Other applications not tested.

Recommended Dilution:
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%). Freeze at -20C or colder. Aliquots are stable for at least 12 months at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
TypeIsotypeCloneGrade
MabIgG1,k3C129Ascites
SizeStorageShippingSourceHost
100ul-20CBlue IceHumanMouse
Concentration:
~1 mg/ml
Immunogen:
CUG-BP1 human nuclear RNA binding protein.
Purity:
Purified by Protein G affinity chromatography.
Form
Supplied as ascites, 0.1% sodium azide.
Specificity:
Specific for human CUG-BP1. Species crossreactivity: Human, mouse, rat, bovine, porcine and rabbit.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Timchenko, L.T., et al. Nucleic Acid Research. 24(22): 4407-4414 (1996). (Immunoblotting)