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t MBNL2 Antibody a

e r o t S Orders: 877-616-CELL (2355) [email protected]

3 Support: 877-678-TECH (8324) 3

7 Web: [email protected]

9 www.cellsignal.com 2

# 3 Trask Lane Danvers Massachusetts 01923 USA For Research Use Only. Not For Use In Diagnostic Procedures.

Applications: Reactivity: Sensitivity: MW (kDa): Source: UniProt ID: - Id: WB H Endogenous 41 Rabbit Q5VZF2 10150 ( ) g g p ( ) g Product Usage Information toxic RNA hairpins that sequester MBNLs in nuclear foci. This loss of available MBNLs causes an adult-to-fetal shift in alternative splicing patterns and ultimately results in Application Dilution respiratory and cardiac complications observed in DM patients (2,5). 1. Pascual, M. et al. (2006) Differentiation 74, 65-80. Western Blotting 1:1000 2. Konieczny, P. et al. (2014) Nucleic Acids Res 42, 10873-87. 3. Lee, K.Y. et al. (2013) EMBO Mol Med 5, 1887-900. Storage 4. Lee, K.S. et al. (2010) J Biol Chem 285, 33779-87. 5. Cho, D.H. and Tapscott, S.J. (2007) Biochim Biophys Acta 1772, 195-204. Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA and 50% glycerol. Store at –20°C. Do not aliquot the antibody. Specificity / Sensitivity MBNL2 Antibody recognizes endogenous levels of total MBNL2 . This antibody does not cross-react with MBNL1 protein. Species Reactivity: Human Source / Purification Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Gly119 of human MBNL2 protein. Antibodies are purified by peptide affinity chromatography. Background Alternative splicing is a crucial biological process that promotes protein diversity and provides cells with an additional mechanism to regulate the expression of tissue-specific protein isoforms. Muscleblind-like (MBNLs) are one such protein family responsible for tissue-specific alternative splicing regulation. MBNLs bind pre-mRNA through an evolutionarily conserved zinc finger domain, and act as either activators or repressors of splicing on specific pre-mRNA targets by promoting the inclusion or exclusion of exons (1). MBNLs are functionally antagonistic to CUG-BP and ETR-3-like factors (CELF proteins) that also control pre-mRNA splicing. The interplay between MBNL and CELF activity plays a key role in development, where predominant MBNL activity promotes adult differentiation, and predominant CELF activity promotes embryonic splicing patterns (1). Three MBNL homologs are expressed in humans (MBNL1, MBNL2, and MBNL3) that share similar structure and function, yet differ in their tissue- and developmental stage-specific expression patterns (2). MBNL1 is the predominant isoform in the majority of tissue, including muscle, and is therefore the most well characterized. MBNL2 is the predominant homolog in brain, and exhibits increased expression upon functional loss of MBNL1 in other tissue types, suggesting a compensatory role (3). MBNL3 appears to play a more specialized role, where it inhibits muscle differentiation in muscle precursor cells (4). Functional loss of MBNLs is observed in myotonic dystrophy (DM), where pathological repeats in the 3’-UTR of the myotonic dystrophy protein kinase (DMPK) gene or intron 1 of the cellular nucleic acid binding protein (CNBP) gene result in

Species reactivity is determined by testing in at least one approved application (e.g., IMPORTANT: For primary antibodies recommended for western blotting applications, western blot). we recommend incubating the membrane with diluted antibody at 4°C with gentle shaking overnight. Please refer to the western blot protocol found on the product web page for the antibody-specific diluent recommendation.

APPLICATIONS KEY WB: Western Blot IP: Immunoprecipitation IHC: Immunohistochemistry ChIP: Chromatin Immunoprecipitation IF: Immunofluorescence F: Flow Cytometry E-P: ELISA-Peptide Cell Signaling Technology is a trademark of Cell Signaling Technology, Inc. Tween is a registered trademark of ICI Americas, Inc. CROSS-REACTIVITY KEY H: human M: mouse R: rat Hm: hamster Mk: monkey Vir: virus Mi: mink C: chicken Dm: D. melanogaster X: Xenopus Z: zebrafish B: bovine Dg: dog Pg: pig Sc: S. cerevisiae Ce: C. elegans Hr: horse All: all species expected https://www.cellsignal.com/datasheet.jsp?productId=29733&images=0&protocol=0 1/2 Revision 1 #29733 MBNL2 Antibody

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CSTLT_86_20200512 Orders: 877-616-CELL (2355) • [email protected] • Support: 877-678-TECH (8324) • [email protected] • Web: www.cellsignal.com https://www.cellsignal.com/datasheet.jsp?productId=29733&images=0&protocol=0 2/2