|Positive WB detected in||Neuro-2a cells, HEK-293 cells, mouse brain tissue, mouse liver tissue, mouse heart tissue, mouse placenta tissue|
|Positive IHC detected in||human pancreas cancer tissue|
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
|Western Blot (WB)||WB : 1:1000-1:4000|
|Immunohistochemistry (IHC)||IHC : 1:20-1:200|
|Sample-dependent, check data in validation data gallery|
13505-1-AP targets ATX3,ATXN3 in WB, IP, IHC, IF, ELISA applications and shows reactivity with human, mouse, rat samples.
|Tested Reactivity||human, mouse, rat|
|Cited Reactivity||C. elegans, human, mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||ATX3,ATXN3 fusion protein Ag4341|
|Full Name||ataxin 3|
|Calculated molecular weight||370 aa, 43 kDa|
|Observed molecular weight||35-42 kDa|
|GenBank accession number||BC033711|
|Gene ID (NCBI)||4287|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.|
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.
Stem Cells Dev
CRISPR/Cas9 targeted deletion of polyglutamine in spinocerebellar ataxia type 3 derived induced pluripotent stem cells.
Mol Biol Cell
A deubiquitinase negatively regulates retro-translocation of nonubiquitinated substrates.
Dis Model Mech
Rescue of ATXN3 neuronal toxicity in Caenorhabditiselegans by chemical modification of endoplasmic reticulum stress.
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.
The Role of the Mammalian DNA End-processing Enzyme Polynucleotide Kinase 3'-Phosphatase in Spinocerebellar Ataxia Type 3 Pathogenesis.
TSPAN8 promotes cancer cell stemness via activation of sonic Hedgehog signaling.