Thermo Fisher Scientific CUGBP1 Polyclonal Antibody

Applications and Tested Dilutions

Product Specifications

Product Specific Information

Antibody detects endogenous levels of total CELF-1.

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, 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. The CUG-BP/hNab50 protein is predominantly nuclear and 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 serves as a binding site for CUG-BP/hNab50, 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.

제품 이미지

(이미지 파일: PA5-101227_CUGBP1_Q92879-1_Rabbit.svg, PA5-101227_CUGBP1_Q92879-1_Rabbit_PDP.jpeg)