Product Info

ELISA 1:10000, WB 1:500-1:2000, IHC 1:200-1:1000, IF/ICC 1:200-1:1000, FCM 1:200-1:400
*The optimal dilutions should be determined by the end user.

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.

Monoclonal [AFB1848]
ATXN1 antibody detects endogenous levels of total ATXN1.
Cite Format: Affinity Biosciences Cat# BF0249, RRID:AB_2833872.
Mouse IgG1 in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.


alternative ataxin1; Ataxin-1; ATX1; ATX1_HUMAN; Atxn1; D6S504E; OTTHUMP00000016065; SCA1; Spinocerebellar ataxia type 1 protein;



Purified recombinant fragment of human ATXN1 expressed in E. Coli.

P54253 ATX1_HUMAN:

Widely expressed throughout the body.

ataxin-1 is a spinocerebellar ataxia type 1 (SCA1) protein. Expansion of CAG repeats in the SCA1 gene results in an abnormally long polyglutamine tract in the protein, causing polyglutamine-induced neurodegeneration. 14-3-3 and Akt kinase stabilize both wild-type and mutant forms of ataxin-1. The expansion of the polyglutamine tract may alter this function.

PTMs - P54253 As Substrate

Site PTM Type Enzyme
K16 Sumoylation
R53 Methylation
S82 Phosphorylation
S88 Phosphorylation
S91 Phosphorylation
K194 Sumoylation
T235 Phosphorylation
S238 Phosphorylation
Y247 Phosphorylation
S253 Phosphorylation
T257 Phosphorylation
S312 Phosphorylation
S366 Phosphorylation
S406 Phosphorylation
S408 Phosphorylation
K609 Sumoylation
K696 Sumoylation
S711 Phosphorylation
S739 Phosphorylation
K745 Sumoylation
T769 Phosphorylation
S775 Phosphorylation P31749 (AKT1) , O75582 (RPS6KA5) , P17612 (PRKACA)
S811 Phosphorylation

Research Backgrounds


Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism. In concert with CIC and ATXN1L, involved in brain development (By similarity).


Ubiquitinated by UBE3A, leading to its degradation by the proteasome. The presence of expanded poly-Gln repeats in spinocerebellar ataxia 1 (SCA1) patients impairs ubiquitination and degradation, leading to accumulation of ATXN1 in neurons and subsequent toxicity.

Phosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract.

Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract.

Subcellular Location:

Cytoplasm. Nucleus.
Note: Colocalizes with USP7 in the nucleus.

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

Widely expressed throughout the body.

Subunit Structure:

Homooligomer. Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBQLN4, ATXN1L and USP7. Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1L for RBPJ-binding. Found in a complex with CIC and ATXN1L (By similarity).


The AXH domain is required for interaction with CIC.

Belongs to the ATXN1 family.

Restrictive clause


Affinity Biosciences tests all products strictly. Citations are provided as a resource for additional applications that have not been validated by Affinity Biosciences. Please choose the appropriate format for each application and consult Materials and Methods sections for additional details about the use of any product in these publications.

For Research Use Only.
Not for use in diagnostic or therapeutic procedures. Not for resale. Not for distribution without written consent. Affinity Biosciences will not be held responsible for patent infringement or other violations that may occur with the use of our products. Affinity Biosciences, Affinity Biosciences Logo and all other trademarks are the property of Affinity Biosciences LTD.