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Mouse Anti-MBNL2 Antibody (3B4) (CBMAB-1419CQ)

This product is a mouse antibody that recognizes MBNL2. The antibody 3B4 can be used for immunoassay techniques such as: WB.
See all MBNL2 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
3B4
Antibody Isotype
IgG2b
Application
WB

Basic Information

Specificity
Human, Mouse
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Supernatant
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
MBNL2
Introduction
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. Diseases associated with MBNL1 include Myotonic Dystrophy and Myotonic Dystrophy 1. Among its related pathways are Adipogenesis. GO annotations related to this gene include poly(A) RNA binding and double-stranded RNA binding.
Entrez Gene ID
Human10150
Mouse105559
UniProt ID
HumanQ5VZF2
MouseQ8C181
Alternative Names
MBLL; MBLL39; PRO2032
Function
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. RNA-binding protein that binds to 5'ACACCC-3' core sequence, termed zipcode, within the 3'UTR of ITGA3. Binds to CUG triplet repeat expansion in myotonic dystrophy muscle cells by sequestering the target RNAs. Seems to regulate expression and localization of ITGA3 by transporting it from the nucleus to cytoplasm at adhesion plaques. May play a role in myotonic dystrophy pathophysiology (DM).
Biological Process
mRNA processing Source: UniProtKB-KW
Regulation of alternative mRNA splicing, via spliceosome Source: GO_Central
Regulation of RNA splicing Source: UniProtKB
RNA splicing Source: UniProtKB-KW
Cellular Location
Cytoplasm
Nucleus
Note: Greater concentration in the nucleus. Expressed in or near large cytoplasmic adhesion plaques (PubMed:16273094). Location in the cytoplasm is microtubule-dependent (PubMed:16273094). In both DM1 and DM2 patients, colocalizes with nuclear foci of retained expanded-repeat transcripts (PubMed:11929853).

Nitschke, L., Hu, R. C., Miller, A. N., Lucas, L., & Cooper, T. A. (2023). Alternative splicing mediates the compensatory upregulation of MBNL2 upon MBNL1 loss-of-function. Nucleic Acids Research, 51(3), 1245-1259.

Wang, L. H., Lin, C. Y., Lin, Y. M., Buée, L., Sergeant, N., Blum, D., ... & Wang, G. S. (2022). Calpain-2 mediates MBNL2 degradation and a developmental RNA processing program in neurodegeneration. Journal of Neuroscience, 42(25), 5102-5114.

Lee, K. Y., Seah, C., Li, C., Chen, Y. F., Chen, C. Y., Wu, C. I., ... & Wang, C. H. (2022). Mice lacking MBNL1 and MBNL2 exhibit sudden cardiac death and molecular signatures recapitulating myotonic dystrophy. Human Molecular Genetics, 31(18), 3144-3160.

Lin, G., Li, J., Cai, J., Zhang, H., Xin, Q., Wang, N., ... & Xu, N. (2021). Rna-Binding protein MBNL2 regulates cancer cell metastasis through mir-182-MBNL2-AKT pathway. Journal of Cancer, 12(22), 6715.

Sta Maria, N. S., Zhou, C., Lee, S. J., Valiulahi, P., Li, X., Choi, J., ... & Reddy, S. (2021). Mbnl1 and Mbnl2 regulate brain structural integrity in mice. Communications Biology, 4(1), 1342.

Cai, J., Wang, N., Lin, G., Zhang, H., Xie, W., Zhang, Y., & Xu, N. (2021). MBNL2 regulates DNA damage response via stabilizing p21. International journal of molecular sciences, 22(02), 783.

Ramon-Duaso, C., Rodríguez-Morató, J., Selma-Soriano, E., Fernández-Avilés, C., Artero, R., de la Torre, R., ... & Robledo, P. (2020). Protective effects of mirtazapine in mice lacking the Mbnl2 gene in forebrain glutamatergic neurons: Relevance for myotonic dystrophy 1. Neuropharmacology, 170, 108030.

Ramon-Duaso, C., Gener, T., Consegal, M., Fernández-Avilés, C., Gallego, J. J., Castarlenas, L., ... & Robledo, P. (2019). Methylphenidate attenuates the cognitive and mood alterations observed in Mbnl2 knockout mice and reduces microglia overexpression. Cerebral Cortex, 29(7), 2978-2997.

Zhang, J., Zheng, Z., Wu, M., Zhang, L., Wang, J., Fu, W., ... & Xu, H. (2019). The natural compound neobractatin inhibits tumor metastasis by upregulating the RNA-binding-protein MBNL2. Cell Death & Disease, 10(8), 554.

André, L. M., van Cruchten, R. T., Willemse, M., & Wansink, D. G. (2019). (CTG) n repeat-mediated dysregulation of MBNL1 and MBNL2 expression during myogenesis in DM1 occurs already at the myoblast stage. PLoS One, 14(5), e0217317.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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