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Product Name
ATX3,ATXN3 antibody
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Description
ATX3,ATXN3 Rabbit Polyclonal antibody. Positive IHC detected in human pancreas cancer tissue. Positive WB detected in mouse pancreas tissue, HEK-293 cells, mouse brain tissue, mouse heart tissue, mouse liver tissue, mouse placenta tissue. Observed molecular weight by Western-blot: 35-42 kDa
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Tested applications
ELISA, WB, IHC
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Species reactivity
Human,Mouse,Rat; other species not tested.
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Alternative names
AT3 antibody; ataxin 3 antibody; ATX3 antibody; ATXN3 antibody; JOS antibody; MJD antibody; MJD1 antibody; SCA3 antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of ATX3,ATXN3 recombinant protein (Accession Number: BC033711). Purification method: Antigen affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:200-1:2000
IHC: 1:20-1:200
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Validations
mouse pancreas tissue were subjected to SDS PAGE followed by western blot with Catalog No:108328(ATX3,ATXN3 antibody) at dilution of 1:500
Immunohistochemical of paraffin-embedded human pancreas cancer using Catalog No:108328(ATX3,ATXN3 antibody) at dilution of 1:100 (under 10x lens)
Immunohistochemical of paraffin-embedded human pancreas cancer using Catalog No:108328(ATX3,ATXN3 antibody) at dilution of 1:100 (under 40x lens)
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Background
ATXN3, which has deubiquitinase activity and act as a component of the ubiquitin proteasome system, plays a role in transcriptional regulation and neuroprotection. ATXN3 interacts with RAD23, HHR23A and HHR23B, involves in the pathology of MJD. ATXN3 is a mixed-linkage, chain-editing enzyme and that the UIM region of ATXN3 regulates its substrate specificity. Contains an N-terminal deubiquitinating domain, called the Josephin domain, followed by 2 ubiquitin-interacting motifs (UIMs) and a polyQ tract near the C terminus. ATXN3 can be phosphorylated in a protein casein kinase-2-dependent manner, thus the MW would be larger than the predicted one.
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References
- Chatterjee A, Saha S, Chakraborty A. The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3'-phosphatase in spinocerebellar ataxia type 3 pathogenesis. PLoS genetics. 11(1):e1004749. 2015.
- Bernardi KM, Williams JM, Inoue T, Schultz A, Tsai B. A deubiquitinase negatively regulates retro-translocation of nonubiquitinated substrates. Molecular biology of the cell. 24(22):3545-56. 2013.
- Switonski PM, Szlachcic WJ, Krzyzosiak WJ, Figiel M. A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD. Neurobiology of disease. 73:174-88. 2015.
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