Applications | Tested Dilution | Publications |
---|---|---|
Western Blot (WB) |
1:2,000 | - |
Immunohistochemistry (Paraffin) (IHC (P)) |
1:50 | - |
Immunocytochemistry (ICC/IF) |
1:100 | - |
Product Specifications | |
---|---|
Species Reactivity |
Human |
Host/Isotype |
Mouse / IgG2b |
Class |
Monoclonal |
Type |
Antibody |
Clone |
OTI5B8 |
Immunogen |
Full length human recombinant protein of human CUGBP1 produced in HEK293T cell. |
Conjugate |
Unconjugated |
Form |
liquid |
Concentration |
0.99 mg/mL |
Purification |
Affinity chromatography |
Storage buffer |
PBS with 1% BSA, 50% glycerol |
Contains |
0.02% sodium azide |
Storage conditions |
-20° C, Avoid Freeze/Thaw Cycles |
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 50 kDa nuclear polyadenylated RNA-binding protein; bruno-like 2; Bruno-like protein 2; 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; CUG-BP- and ETR-3-like factor 1; CUG-BP1; CUGBP Ela; CUGBP Elav-like family member 1; Deadenylation factor CUG-BP; EDEN-BP homolog; embryo deadenylation element binding protein; Embryo deadenylation element-binding protein homolog; nuclear polyadenylated RNA-binding protein, 50-kD; RNA-binding protein BRUNOL-2; RP23-147D3.7
Gene Aliases: BRUNOL2; CELF1; CUG-BP; CUGBP; CUGBP1; EDEN-BP; hNab50; NAB50; NAPOR
UniProt ID: (Human) Q92879
Entrez Gene ID: (Human) 10658
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