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Mammalian Muscleblind Proteins (Clone MBNL)

Mouse Monoclonal Antibody

PRODUCT #: 40-1270

 

DESCRIPTION: Muscleblind was originally isolated following studies of Drosophila, since inactivation of the muscleblind (mbl) gene in this species resulted in defects in the development of muscles and the visual system.  The Drosophila muscleblind protein contains 4 zinc finger nucleic acid binding motifs. These binding domains are about 26 amino-acids long, co-ordinate one zinc ion and bind RNA.  Human homologues of the single Drosophila muscleblind gene were discovered from cDNA and genomic sequencing, but were also discovered as a result of experiments aimed at finding proteins which bind to polynucleotide repeated sequences.  Several important human diseases are associated with expansion of polynucleotide sequences, in most cases trinucleotide repeats. Myotonic dystrophy (DM1) is one of these diseases, and is associated with increases in the number of CTG repeats in the 3' UTR of the gene encoding myotonin (a.k.a. DM Kinase, DMK or myotonin-Protein Kinase), a ser/thr kinase expressed specifically in muscle.
   
SPECIES CROSS-REACTIVITY: Human, mouse, rat

 

APPLICATION/DILUTIONS:        ICC/IF (1/50 - 1/2,000)
WB (1/500 – 1/2,000)
   
SOURCE: Mice were immunized with full-length recombinant human MBNL expressed in E. coli.
   
FORM/STORAGE: 0.1 ml with 50% glycerol, 0.01% sodium azide. 
Store at -20º C.  Avoid multiple freeze/thaw cycles. 
   
Reference: Begemann G. et al. Development 124:4321-4331 (1997).
   
 
THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN HUMANS OR DIAGNOSTIC PROCEDURES.