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Rabbit Anti-ATXN3 Recombinant Antibody (A4164) (CBMAB-A4164-YC)

Provided herein is a Mouse monoclonal antibody against Human Ataxin 3. The antibody can be used for immunoassay techniques, such as ELISA, FC, IHC-P, WB.
See all ATXN3 antibodies
Published Data
Rabbit Anti-ATXN3 Recombinant Antibody (A4164) in WB
As assay controls, ataxin-3-mediated breakdown of K63-linked polyubiquitin chains and of lmw, medium-molecular (mmw) and hmw pUb forms of p53 were detected. Addition of His6-ataxin-3 was confirmed by immunodetection. GAPDH served as loading control. ...View More
Harmuth, T., Weber, J. J., Zimmer, A. J., Sowa, A. S., Schmidt, J., Fitzgerald, J. C., ... & Hübener-Schmid, J. (2022). Mitochondrial dysfunction in spinocerebellar ataxia type 3 is linked to VDAC1 deubiquitination. International Journal of Molecular Sciences, 23(11), 5933.

Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
A4164
Antibody Isotype
IgG
Application
WB, IF, IP

Basic Information

Immunogen
Protein corresponding to human Ataxin 3 (aa1-aa364).
Specificity
Human, Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB2.5 µg/ml
IF(ICC)5 µg/ml

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

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
0.09% sodium azide
Concentration
0.5 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.
Epitope
aa 112-249

Target

Full Name
Ataxin 3
Introduction
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86
Entrez Gene ID
Human4287
Mouse110616
Rat60331
Monkey720515
UniProt ID
HumanP54252
MouseQ9CVD2
RatO35815
MonkeyH2R2I4
Alternative Names
Ataxin 3; Spinocerebellar Ataxia Type 3 Protein; Machado-Joseph Disease Protein 1; SCA3; ATX3; MJD1; MJD; Machado-Joseph Disease (Spinocerebellar Ataxia 3, Olivopontocerebellar Ataxia 3, Autosomal Dominant, Ataxin 3);
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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