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Invitrogen™ CUGBP1 Recombinant Rabbit Monoclonal Antibody (K01_2L99)

Description
CUGBP1 Recombinant Monoclonal Antibody for ICC/IF, IHC (P), Western Blot
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. 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.
Specifications
Specifications
| Antigen | CUGBP1 |
| Applications | Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry |
| Classification | Recombinant Monoclonal |
| Clone | K01_2L99 |
| Concentration | 1 mg/mL |
| Conjugate | Unconjugated |
| Formulation | 50mM tris glycine with 40% glycerol, 150mM NaCl, 0.05% BSA and 0.01% sodium azide; pH 7.4 |
| Gene | CELF1 |
| Gene Accession No. | Q4QQT3, Q92879 |
| Gene Alias | 1600010O03Rik; 50 kDa nuclear polyadenylated RNA-binding protein; AA407467; Brain protein F41; Brunol2; bruno-like 2; bruno-like protein 2; Celf1; CELF-1; CUG RNA-binding protein; CUG triplet repeat RNA-binding protein 1; CUG triplet repeat, RNA binding protein 1; CUG triplet repeat, RNA-binding protein 1; Cugbp; CUG-BP; CUG-BP- and ETR-3-like factor 1; CUGBP Ela; CUGBP Elav-like family member 1; CUGBP, Elav-like family member 1; CUGBP, Elav-like family member 1-like; CUGBP1; CUG-BP1; D2Wsu101e; Deadenylation factor CUG-BP; deadenylation factor EDEN-BP; EDEN-BP; EDEN-BP homolog; embryo deadenylation element binding protein; embryo deadenylation element-binding protein homolog; HNAB50; NAB50; NAPOR; nuclear polyadenylated RNA-binding protein, 50-kD; RNA-binding protein BRUNOL-2; RP23-147D3.7 |
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Titolo del prodotto
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