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Mouse Anti-ATXN3 Recombinant Antibody (B-10) (CBMAB-0290-WJ)

This product is a mouse antibody that recognizes ATXN3. The antibody B-10 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all ATXN3 antibodies
Published Data
Mouse Anti-ATXN3 Recombinant Antibody (B-10) in Immunoblot
P03 cells were treated with different concentrations of GY1-22 for 24 h and subjected to immunoprecipitation by anti-DNAJA1 antibody, followed by Western blotting to detect mutp53, ataxin-3, and DNAJA1 protein levels. ...View More
Tong, X., Xu, D., Mishra, R. K., Jones, R. D., Sun, L., Schiltz, G. E., ... & Yang, G. Y. (2021). Identification of a druggable protein-protein interaction site between mutant p53 and its stabilizing chaperone DNAJA1. Journal of Biological Chemistry, 296.

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
B-10
Antibody Isotype
IgG2b, κ
Application
WB, IP, IF, ELISA, IHC-P

Basic Information

Immunogen
Amino acids 176-234 of Ataxin-3 of mouse origin.
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
ELISA1:100-1:1,000
IF(ICC)1:50-1:500
IHC-P1:50-1:500

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

Format
Liquid
Buffer
PBS, 0.1% gelatin
Preservative
< 0.1% sodium azide
Concentration
0.2 mg/ml
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
Ataxin 3
Introduction
ATXN3 (Ataxin 3) encodes the protein which contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. Diseases associated with ATXN3 include Machado-Joseph Disease and Machado-Joseph Disease Type 1. An important paralog of this gene is ATXN3L.
Entrez Gene ID
4287
UniProt ID
P54252
Alternative Names
AT3; JOS; MJD; ATX3; MJD1; SCA3
Function
Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278).
Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed:17696782).
Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (By similarity).
Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (PubMed:12297501).
Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460).
Biological Process
Actin cytoskeleton organization Source: MGI
Cellular response to heat Source: ParkinsonsUK-UCL
Cellular response to misfolded protein Source: UniProtKB
Chemical synaptic transmission Source: ProtInc
Intermediate filament cytoskeleton organization Source: MGI
Microtubule cytoskeleton organization Source: MGI
Monoubiquitinated protein deubiquitination Source: UniProtKB
Nervous system development Source: ProtInc
Nucleotide-excision repair Source: ProtInc
Positive regulation of ERAD pathway Source: ParkinsonsUK-UCL
Proteasome-mediated ubiquitin-dependent protein catabolic process Source: UniProtKB
Protein deubiquitination Source: ParkinsonsUK-UCL
Protein K48-linked deubiquitination Source: ParkinsonsUK-UCL
Protein K63-linked deubiquitination Source: ParkinsonsUK-UCL
Protein localization to cytosolic proteasome complex involved in ERAD pathway Source: ParkinsonsUK-UCL
Protein quality control for misfolded or incompletely synthesized proteins Source: UniProtKB
Regulation of cell-substrate adhesion Source: MGI
Ubiquitin-dependent protein catabolic process Source: ParkinsonsUK-UCL
Cellular Location
Nucleus matrix; Nucleus. Predominantly nuclear, but not exclusively, inner nuclear matrix.
Involvement in disease
Spinocerebellar ataxia 3 (SCA3): Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATX3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
PTM
Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (PubMed:23696636).

Herzog, L. K., Kevei, É., Marchante, R., Böttcher, C., Bindesbøll, C., Lystad, A. H., ... & Dantuma, N. P. (2020). The Machado–Joseph disease deubiquitylase ataxin‐3 interacts with LC3C/GABARAP and promotes autophagy. Aging Cell, 19(1), e13051.

Toulis, V., García-Monclús, S., de la Peña-Ramírez, C., Arenas-Galnares, R., Abril, J. F., Todi, S. V., ... & Marfany, G. (2020). The deubiquitinating enzyme ataxin-3 regulates ciliogenesis and phagocytosis in the retina. Cell Reports, 33(6), 108360.

Singh, B., Bose, P., Shamal, S. N., Joshi, D., & Singh, R. (2020). Polymorphic study of ataxin 3 gene in Eastern Uttar Pradesh population. Journal of The Anatomical Society of India, 69(4), 226.

Blount, J. R., Johnson, S. L., Libohova, K., Todi, S. V., & Tsou, W. L. (2020). Degron capability of the hydrophobic C‐terminus of the polyglutamine disease protein, ataxin‐3. Journal of Neuroscience Research, 98(10), 2096-2108.

Weishäupl, D., Schneider, J., Pinheiro, B. P., Ruess, C., Dold, S. M., von Zweydorf, F., ... & Schmidt, T. (2019). Physiological and pathophysiological characteristics of ataxin-3 isoforms. Journal of Biological Chemistry, 294(2), 644-661.

McIntosh, C. S., Aung-Htut, M. T., Fletcher, S., & Wilton, S. D. (2019). Removal of the polyglutamine repeat of Ataxin-3 by redirecting pre-mRNA processing. International journal of molecular sciences, 20(21), 5434.

Johnson, S. L., Blount, J. R., Libohova, K., Ranxhi, B., Paulson, H. L., Tsou, W. L., & Todi, S. V. (2019). Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3. Neurobiology of disease, 132, 104535.

Jatho-Gröger, J. (2018). Deciphering the role of the deubiquitinating enzyme Ataxin-3 in transcriptional regulation and the cellular response to stress.

Marfany, G., Toulis, V., & do Carmo Costa, M. (2017). The deubiquitinating enzyme Ataxin-3 is relevant for retina formation and differentiation in mouse and zebrafish. Investigative Ophthalmology & Visual Science, 58(8), 599-599.

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

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