Ataxin 3 Antibody - #DF6375

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Product: Ataxin 3 Antibody
Catalog: DF6375
Description: Rabbit polyclonal antibody to Ataxin 3
Application: WB IHC
Reactivity: Human, Mouse, Rat
Prediction: Pig, Zebrafish, Bovine, Horse, Sheep, Rabbit, Dog, Chicken, Xenopus
Mol.Wt.: 43kDa; 41kD(Calculated).
Uniprot: P54252
RRID: AB_2838339

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Related Downloads
MSDS
Data Sheet
COA
Protocols
WB Handbook
IHC Protocol
IF/ICC Protocol

Product Info

Source:
Rabbit
Application:
WB 1:500-1:2000, IHC 1:50-1:200
*The optimal dilutions should be determined by the end user.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

Reactivity:
Human,Mouse,Rat
Prediction:
Pig(100%), Zebrafish(86%), Bovine(100%), Horse(100%), Sheep(100%), Rabbit(100%), Dog(100%), Chicken(100%), Xenopus(100%)
Clonality:
Polyclonal
Specificity:
Ataxin 3 Antibody detects endogenous levels of total Ataxin 3.
RRID:
AB_2838339
Cite Format: Affinity Biosciences Cat# DF6375, RRID:AB_2838339.
Conjugate:
Unconjugated.
Purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
Storage:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
Alias:

Fold/Unfold

AT3; Ataxin 3; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; Ataxin-3; ATX3; ATX3_HUMAN; ATXN3; EC 3.4.22.; JOS; Josephin; Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); Machado Joseph disease; Machado Joseph disease protein 1; Machado-Joseph disease protein 1; Machado-Joseph disease protein 1 homolog; MJD; MJD gene; MJD1; Olivopontocerebellar ataxia 3; OTTHUMP00000221583; OTTHUMP00000221585; OTTHUMP00000221586; OTTHUMP00000221587; OTTHUMP00000231995; OTTHUMP00000231997; Rsca3; SCA3; SCA3 gene; Spinocerebellar ataxia type 3 protein;

Immunogens

Immunogen:
Uniprot:

P54252(ATX3_HUMAN) >>Visit HPA database.

Gene(ID):
ATXN3(4287)
Expression:
P54252 ATX3_HUMAN:

Ubiquitous.

Description:
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Sep 2009]
Sequence:
MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERMRMAEGGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQRLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADLRRAIQLSMQGSSRNISQDMTQTSGTNLTSEELRKRREAYFEKQQQKQQQQQQQQQQGDLSGQSSHPCERPATSSGALGSDLGDAMSEEDMLQAAVTMSLETVRNDLKTEGKK

Predictions

Predictions:

Score>80(red) has high confidence and is suggested to be used for WB detection. *The prediction model is mainly based on the alignment of immunogen sequences, the results are for reference only, not as the basis of quality assurance.

Species
Results
Score
Pig
100
Horse
100
Bovine
100
Sheep
100
Dog
100
Xenopus
100
Chicken
100
Rabbit
100
Zebrafish
86
Model Confidence:
High(score>80) Medium(80>score>50) Low(score<50) No confidence

PTMs - P54252 As Substrate

Site PTM Type Enzyme
K8 Ubiquitination
S12 Phosphorylation
S55 Phosphorylation
Y58 Phosphorylation
T60 Phosphorylation
K117 Sumoylation
K117 Ubiquitination
K166 Sumoylation
K190 Ubiquitination
K200 Ubiquitination
K206 Ubiquitination
S219 Phosphorylation
S236 Phosphorylation
S256 Phosphorylation P49841 (GSK3B)
S260 Phosphorylation
S265 Phosphorylation
T269 Phosphorylation
S278 Phosphorylation
K291 Ubiquitination
S335 Phosphorylation
S347 Phosphorylation
K360 Acetylation

Research Backgrounds

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 (By similarity). Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription. Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1.

PTMs:

Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity.

Subcellular Location:

Nucleus matrix. Nucleus.
Note: Predominantly nuclear, but not exclusively, inner nuclear matrix.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
Tissue Specificity:

Ubiquitous.

Subunit Structure:

Interacts with STUB1/CHIP (when monoubiquitinated) (By similarity). Interacts with DNA repair proteins RAD23A and RAD23B. Interacts with BECN1 (via its poly-Gln domain). Interacts with PRKN, UBR2, VCP and tubulin. Short isoform 1 interacts with CASP7.

Family&Domains:

The UIM domains bind ubiquitin and interact with various E3 ubiquitin-protein ligase, such as STUB1/CHIP. They are essential to limit the length of ubiquitin chains (By similarity).

The poly-Gln domain is involved in the interaction with BECN1 and subsequent starvation-induced autophagy (PubMed:28445460).

Research Fields

· Genetic Information Processing > Folding, sorting and degradation > Protein processing in endoplasmic reticulum.   (View pathway)

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