Muscleblind-like protein 1; Triplet-expansion RNA-binding protein; MBNL1; EXP; KIAA0428; MBNL

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This MBNL1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 255-284 amino acids from the C-terminal region of human MBNL1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-MBNL1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU(U/G)Y-3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues.

Western Blot (WB), ELISA (EIA)

WB~~1:1000

MBNL1 Antibody (C-term) western blot analysis in Jurkat cell line lysates (35ug/lane).This demonstrates the MBNL1 antibody detected the MBNL1 protein (arrow).

41817

muscleblind-like splicing regulator 1

muscleblind-like protein 1

Muscleblind-like protein 1

EXP; KIAA0428; MBNL??[Similar Products]

MBNL1_HUMAN

MBNL1: Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU(U/G)Y- 3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues. Plays a role in the pathogenesis of dystrophia myotonica type 1 (DM1). A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. In muscle cells from DM1 patients, MBNL1 is sequestered by DMPK RNAs containing CUG triplet repeat expansions. MBNL1 binding is proportional to repeat length consistent with the direct correlation between the length of repeat expansion and disease severity. Belongs to the muscleblind family. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA-binding; RNA splicing

Chromosomal Location of Human Ortholog: 3q25

Cellular Component: nucleoplasm; centrosome; stress granule; cytoplasm; nucleus

Molecular Function: protein binding; RNA binding; metal ion binding; double-stranded RNA binding

Biological Process: myoblast differentiation; alternative nuclear mRNA splicing, via spliceosome; mRNA splice site selection; nervous system development; skeletal muscle development; regulation of RNA splicing; in utero embryonic development; RNA splicing; regulation of alternative nuclear mRNA splicing, via spliceosome; embryonic limb morphogenesis

Sen, S., et al. J. Biol. Chem. 285(33):25426-25437(2010)
Arambula, J.F., et al. Proc. Natl. Acad. Sci. U.S.A. 106(38):16068-16073(2009)
Cardani, R., et al. Neuromuscul. Disord. 19(5):335-343(2009)
Holt, I., et al. Am. J. Pathol. 174(1):216-227(2009)
Teplova, M., et al. Nat. Struct. Mol. Biol. 15(12):1343-1351(2008)

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