CELF1 Mouse Monoclonal Antibody [7A1]
Myotonic dystrophy (DM) 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 gene (DMPK). CUG-BP1 and CUG-BP2 are proteins that bind specifically to (CUG)8 oligonucleotides in vitro. While CUG-BP1 has the major binding activity in normal cells, nuclear CUG-BP2 binding activity increases in DM cells. Both CUG-BP1 and CUG-BP2 are isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50. CUG-BP1, an RNA CUG triplet repeat binding protein, regulates splicing and translation of various RNAs. Expansion of RNA CUG repeats in the DMPK in DM is associated with alterations in binding activity of CUG-BP1 as well as alterations in the translation of the C/EBPb transcription factor. CUG-BP1 is an important regulator of initiation from different AUG codons of C/EBPb mRNA. In normal cells, CUG-BP1 up-regulates the p21 protein during differentiation by inducing the translation of p21 via binding to a GC-rich sequence located within the 5' region of p21 mRNA. In DM cells, failure to accumulate CUG-BP1 leads to a reduction of p21 and alterations in other proteins responsible for cell cycle withdrawl.
Product Specifications
Product Name Alternative
Abbreviation
CELF-1, CUG-BP1, EDEN-BP homolog, CELF1, BRUNOL2, CUGBP, CUGBP1, NAB50
Swiss Prot
Q92879 Human, P28659 Mouse, Q4QQT3 Rat
Cellular Locus
Cytoplasm. Nucleus.
Host
Mouse
Species Reactivity
Human,Mouse,Rat
Immunogen
Recombinant protein within Human CELF1 aa 1-200 / 486.
Isotype
IgG1
Conjugation
Non-conjugated
Type
Mouse monoclonal Antibody
Applications
WB, IHC-P, IF-Cell
Positive Control
Concentration
2 mg/mL
Dilution
WB: 1:2,000-1:5,000 ;IHC-P: 1:1,000 ;IF-Cell: 1:100
Purity
Immunogen affinity purified.
Form
Liquid
Buffer
1*PBS (pH7.4), 0.2% BSA, 50% Glycerol. Preservative: 0.05% Sodium Azide.
Molecular Weight
Predicted band size: 52 kDa
Storage Conditions
Store at +4°C after thawing. Aliquot store at -20°C. Avoid repeated freeze/thaw cycles.
Recombinant Antibody
No
CAS Number
9007-83-4
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