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|Tested species reactivity||Non-human primate, Pig, Rabbit, Rat, Bovine, Human, Mouse|
|Host / Isotype||Mouse / IgG1, kappa|
|Immunogen||CUG-BP1 human nuclear RNA binding protein.|
|Storage buffer||tris glycine with 150mM NaCl|
|Contains||0.02% sodium azide|
|Storage Conditions||-20° C, Avoid Freeze/Thaw Cycles|
|Tested Applications||Dilution *|
|Flow Cytometry (Flow)||1 µg/10^6 cells|
|Gel Shift (GS)||Assay-Dependent|
|Immunohistochemistry (Paraffin) (IHC (P))||1:100-1:500|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
Suggested positive control: antigen standard for CUGBP1 (transient overexpression lysate).
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.
1600010O03Rik; 50 kDa nuclear polyadenylated RNA-binding protein; AA407467; Brain protein F41; bruno-like 2; Bruno-like protein 2; Brunol2; 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; CUG-BP- and ETR-3-like factor 1; CUG-BP1; CUGBP; CUGBP Elav-like family member 1; CUGBP, Elav-like family member 1; D2Wsu101e; Deadenylation factor CUG-BP; Deadenylation factor EDEN-BP; EDEN-BP homolog; embryo deadenylation element binding protein; Embryo deadenylation element-binding protein homolog; HNAB50; NAB50; nuclear polyadenylated RNA-binding protein, 50-kD; RNA-binding protein BRUNOL-2; RP23-147D3.7
1600010O03Rik; AA407467; BOS_15139; BRUNOL2; CELF1; CUG-BP; CUG-BP1; CUGBP; CUGBP1; D2Wsu101e; EDEN-BP; HNAB50; NAB50; NAPOR