Anti-Human ATXN3 Polyclonal Antibody

Catalog No.

PHF02401

Species reactivity

Human

Host species

Rabbit

Isotype

IgG

Clonality

Polyclonal

Immunogen

E. coli - derived recombinant Human ATXN3 (Met1-Arg182).

Tested applications

ELISA: 1:4000-1:8000, IHC: 1:50-1:100, WB: 1:1000-1:4000

Target

ATX3, MJD1, MJD, SCA3, Ataxin-3, Spinocerebellar ataxia type 3 protein, ATXN3, Machado-Joseph disease protein 1

Purification

Purified by antigen affinity column.

Accession

P54252

Applications

ELISA, IHC, WB

Form

Liquid

Storage buffer

0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300.

Stability and Storage

Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.

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

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