CUGBP1 Mouse anti-Human, Clone: OTI5B8, liquid, TrueMAB
Mouse Monoclonal Antibody
Manufacturer: OriGene TA500951S
DescriptionMyotonic dystrophy (MD) is an autosomal domit neuromuscular disease that is associated with a (CTG)n repeat expansion in the 3 ft.-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 predomitly 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.
|PBS with 1% BSA, 50% glycerol and 0.02% sodium azide|
|BRUNOL2, CUG-BP, CUGBP, CUGBP1, EDEN-BP, NAB50, NAPOR, hNab50|
|-20° C, Avoid Freeze/Thaw Cycles|
|Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot|
|Full length human recombit protein of human CUGBP1 produced in HEK293T cell.|
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