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MBNL3 Antibody [PCRP-MBNL3-1D11]

In Stock
HuProt Validated
Catalog Number Formulation Size Price
55796-MSM1-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$229.00
55796-MSM1-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$519.00
55796-MSM1-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$519.00
Flat Rate Domestic: $60 | Orders outside the US - Contact Us for Order Information | Ships next business day

Applications & Dilutions

Applications Tested Dillution Protocol
Flow Cytometry (Flow)
1-2ug/million cells

Summary

Pre-mRNA splicing is a critical step in the posttranscriptional regulation of gene expression. Several protein complexes are involved in proper mRNA splicing and transport. The muscleblind proteins, MBNL1, MBNL2 and MBNL3, promote inclusion or exclusion of specific exons on different pre-mRNAs by antagonizing the activity of CUG-BP and ETR-3-like factors bound to distinct intronic sites. MBNL1 and 2, which associate with expanded CUG repeats in vitro, localize to the nuclear foci in both DM1 and DM2 (myotonic dystrophy types 1 and 2), suggesting that the nuclear accumulation of mutant RNA is pathogenic in DM1, therefore implicating MBNL1 and 2 in the pathogenesis of both disorders. MBNL3, a 354 amino acid protein, inhibits expression of muscle differentiation, opposite to the function of MBNL1, which functions as a promoter of muscle differentiation. MBNL3 shows strong expression in placenta.

Product Properties & Targets

Antibody Type
Host
Mouse
Applications
Species Reactivity
Research Areas
Isotype / Light Chain
IgG2b /
Cellular Localization
Cytoplasm, Nucleus
Gene Name
Positive Control
HeLa or MCF7 cells.
Immunogen
Recombinant full-length human MBNL3 protein
Alternate Names
Muscleblind-like protein 3, Cys3His CCG1-required protein, Muscleblind-like X-linked protein, Protein HCHCR, CHCR; Cys3His CCG1 required protein; MBLX; MBLX39; Muscleblind like protein 3; Muscleblind like splicing regulator 3; Muscleblind-like X-linked protein; Protein HCHCR; RP23-98P13.1

Database Links

Entrez Gene ID
SwissProt

Additional Information

Clone
PCRP-MBNL3-1D11
Chromosome Location
Xq26.2
Mol. Weight of Antigen
39kDa

Functions

  • 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. May play a role in myotonic dystrophy pathophysiology (DM). Could inhibit terminal muscle differentiation, acting at approximately the time of myogenin induction.

Key References

  • Ishikawa, K., et al. 1998. DNA Res. 4: 307-313.
  • Ishiura, S. 2005. Molecular pathways to myotonic dystrophy. Nippon Rinsho 63: 515-521.

Storage & Stability

Antibody with azide - store at 2 to 8 °C. Antibody without azide - store at -20 to -80 °C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Limitations

This antibody is available for research use only and is not approved for use in diagnosis.

Supplied as

200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Warranty

There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.

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