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

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

Natural and recombinant Human Muscleblind-like protein 1

For long term storage, please store the entire kit at -20 degree C.

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Small volumes of MBNL1 elisa kit 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.

MBS2890313 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the MBNL1/Muscleblind-Like Protein 1, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MBNL1. The ELISA analytical biochemical technique of the MBS2890313 kit is based on MBNL1 antibody-MBNL1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MBNL1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MBNL1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

muscleblind like splicing regulator 1

muscleblind-like protein 1

Muscleblind-like protein 1

EXP; KIAA0428; MBNL??[Similar Products]

MBNL1_HUMAN

This gene encodes a member of the muscleblind protein family which was initially described in Drosophila melanogaster. The encoded protein is a C3H-type zinc finger protein that modulates alternative splicing of pre-mRNAs. Muscleblind proteins bind specifically to expanded dsCUG RNA but not to normal size CUG repeats and may thereby play a role in the pathophysiology of myotonic dystrophy. Mice lacking this gene exhibited muscle abnormalities and cataracts. Several alternatively spliced transcript variants have been described but the full-length natures of only some have been determined. The different isoforms are thought to have different binding specificities and/or splicing activities. [provided by RefSeq, Sep 2015]

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

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