Anti-ATXN3 antibody (AA23963)

$187.00


Alternative names Ataxin-3, EC 3.4.19.12, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3, ATX3_HUMAN
Species Reactivity Human, Mouse, Rat
Applications IF/ICC, IHC, WB
Host Rabbit
Conjugated Unconjugated


Description

Product name Anti-ATXN3 antibody (AA23963)
Catalog number AA23963
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Ataxin-3 antibody, EC 3.4.19.12 antibody, Machado-Joseph disease protein 1 antibody, Spinocerebellar ataxia type 3 protein antibody, ATXN3 antibody, ATX3 antibody, MJD antibody, MJD1 antibody, SCA3 antibody, ATX3_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ATXN3
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Purification method Purified by affinity chromatography


Target

Target Name ATXN3
SwissProt ID Link P54252
Function Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. 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. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription.
Subcellular Location Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
Involvement in Disease Spinocerebellar ataxia 3: 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 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. The disease is caused by mutations affecting the gene represented in this entry.
Post-Translational Modification Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity.
Tissue Specificity Ubiquitous.
Subunit Structure Interacts with STUB1/CHIP (when monoubiquitinated). Interacts with DNA repair proteins RAD23A and RAD23B.
Sequence Similarities Contains 1 Josephin domain. Contains 3 UIM (ubiquitin-interacting motif) domains.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.