Catalog No.
RHF02401
Species reactivity
Human
Host species
Rabbit
Isotype
IgG
Clonality
Monoclonal
Tested applications
WB: 1:500-1:1000
Target
ATX3, MJD1, MJD, SCA3, Ataxin-3, Spinocerebellar ataxia type 3 protein, ATXN3, Machado-Joseph disease protein 1
Concentration
1 mg/ml
Endotoxin level
Please contact with the lab for this information.
Purity
>95% by SDS-PAGE.
Purification
Protein A/G purified from cell culture supernatant.
Accession
P54252
Applications
WB
Form
Liquid
Storage buffer
0.01M PBS, pH 7.4, 0.05% BSA, 50% Glycerol, 0.05% Sodium azide.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 4 ℃ for frequent use. Store at -20 ℃ for twelve months from the date of receipt.
Clone ID
R1Y86
Accurate Quantification of Mutant and Wild-Type polyQ Proteins Using Simple Western Capillary Immunoassays., PMID:40450087
VCP/p97-associated proteins are binders and debranching enzymes of K48-K63-branched ubiquitin chains., PMID:38977901
CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration., PMID:24548080
Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3., PMID:23617879
Usp14 deficiency increases tau phosphorylation without altering tau degradation or causing tau-dependent deficits., PMID:23144711
A major role for side-chain polyglutamine hydrogen bonding in irreversible ataxin-3 aggregation., PMID:21533208
Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels., PMID:20668528
Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity., PMID:19492089
Differential effects of polyglutamine proteins on nuclear organization and artificial reporter splicing., PMID:17526020
Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3., PMID:17488727
Towards a structural understanding of the fibrillization pathway in Machado-Joseph's disease: trapping early oligomers of non-expanded ataxin-3., PMID:16194547
Misfolding promotes the ubiquitination of polyglutamine-expanded ataxin-3, the defective gene product in SCA3/MJD., PMID:15639784
A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration., PMID:15537899
Amyotrophic lateral sclerosis with neuronal intranuclear protein inclusions., PMID:15114487
Intranuclear aggregation of nonexpanded ataxin-3 in marinesco bodies of the nonhuman primate substantia nigra., PMID:12093088
Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains., PMID:10993685
Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix., PMID:10556285
Heterogeneous intracellular localization and expression of ataxin-3., PMID:10069576
An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients., PMID:9804376
Ataxin-3 is transported into the nucleus and associates with the nuclear matrix., PMID:9580663
Machado-Joseph disease gene product identified in lymphocytes and brain., PMID:9144561
For research use only. Not for human or drug use.
