Synthetic peptide directed towards the middle region of human MBNL2
Application
Anti-MBNL2 antibody produced in rabbit is suitable for western blotting at a concentration of 0.5 μg/ml.
Biochem/physiol Actions
Muscleblind-like splicing regulator 2 (MBNL2) is a C3H-type zinc finger protein that modulates the alternate splicing of pre-mRNA molecules. MBNL2-mediated splicing program is essential in the developing brain. The dysregulation of MBNL2 splicing activity results in myotonic dystrophy.
Sequence
Synthetic peptide located within the following region: ENGRVIACFDSLKGRCSRENCKYLHPPTHLKTQLEINGRNNLIQQKTAAA
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
The RNA-mediated disease model for myotonic dystrophy (DM) proposes that microsatellite C(C)TG expansions express toxic RNAs that disrupt splicing regulation by altering MBNL1 and CELF1 activities. While this model explains DM manifestations in muscle, less is known about the effects
The American journal of pathology, 174(1), 216-227 (2008-12-20)
In myotonic dystrophy, muscleblind-like protein 1 (MBNL1) protein binds specifically to expanded CUG or CCUG repeats, which accumulate as discrete nuclear foci, and this is thought to prevent its function in the regulation of alternative splicing of pre-mRNAs. There is
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