|Tested species reactivity||Bovine, Human, Mouse, Non-human primate, Pig, Rabbit, Rat|
|Published species reactivity||Not Applicable|
|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.
|Miscellaneous PubMed (MISC)||See 1 publications below|
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.
|Not Applicable||Not Cited||
Analysis of MTMR1 expression and correlation with muscle pathological features in juvenile/adult onset myotonic dystrophy type 1 (DM1) and in myotonic dystrophy type 2 (DM2).
MA1-16675 was used in immunohistochemistry and western blot to investigate the relationship between MTMR1 expression and CUG-BP1 levels in myotonic dystrophy 1 and 2 patients
|Santoro M,Modoni A,Masciullo M,Gidaro T,Broccolini A,Ricci E,Tonali PA,Silvestri G||Experimental and molecular pathology (89:158)||2010|