ATXN1 Antibody - #BF0249
製品説明
*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.
引用形式: Affinity Biosciences Cat# BF0249, RRID:AB_2833872.
折りたたみ／展開
alternative ataxin1; Ataxin-1; ATX1; ATX1_HUMAN; Atxn1; D6S504E; OTTHUMP00000016065; SCA1; Spinocerebellar ataxia type 1 protein;
免疫原
Purified recombinant fragment of human ATXN1 expressed in E. Coli.
- P54253 ATX1_HUMAN:
- Protein BLAST With
- NCBI/
- ExPASy/
- Uniprot
MKSNQERSNECLPPKKREIPATSRSSEEKAPTLPSDNHRVEGTAWLPGNPGGRGHGGGRHGPAGTSVELGLQQGIGLHKALSTGLDYSPPSAPRSVPVATTLPAAYATPQPGTPVSPVQYAHLPHTFQFIGSSQYSGTYASFIPSQLIPPTANPVTSAVASAAGATTPSQRSQLEAYSTLLANMGSLSQTPGHKAEQQQQQQQQQQQQHQHQQQQQQQQQQQQQQHLSRAPGLITPGSPPPAQQNQYVHISSSPQNTGRTASPPAIPVHLHPHQTMIPHTLTLGPPSQVVMQYADSGSHFVPREATKKAESSRLQQAIQAKEVLNGEMEKSRRYGAPSSADLGLGKAGGKSVPHPYESRHVVVHPSPSDYSSRDPSGVRASVMVLPNSNTPAADLEVQQATHREASPSTLNDKSGLHLGKPGHRSYALSPHTVIQTTHSASEPLPVGLPATAFYAGTQPPVIGYLSGQQQAITYAGSLPQHLVIPGTQPLLIPVGSTDMEASGAAPAIVTSSPQFAAVPHTFVTTALPKSENFNPEALVTQAAYPAMVQAQIHLPVVQSVASPAAAPPTLPPYFMKGSIIQLANGELKKVEDLKTEDFIQSAEISNDLKIDSSTVERIEDSHSPGVAVIQFAVGEHRAQVSVEVLVEYPFFVFGQGWSSCCPERTSQLFDLPCSKLSVGDVCISLTLKNLKNGSVKKGQPVDPASVLLKHSKADGLAGSRHRYAEQENGINQGSAQMLSENGELKFPEKMGLPAAPFLTKIEPSKPAATRKRRWSAPESRKLEKSEDEPPLTLPKPSLIPQEVKICIEGRSNVGK
PTMs - P54253 基板として
| Site | PTM Type | Enzyme | Source |
|---|---|---|---|
| K16 | Sumoylation | Uniprot | |
| R53 | Methylation | Uniprot | |
| S82 | Phosphorylation | Uniprot | |
| S88 | Phosphorylation | Uniprot | |
| S91 | Phosphorylation | Uniprot | |
| K194 | Sumoylation | Uniprot | |
| T235 | Phosphorylation | Uniprot | |
| S238 | Phosphorylation | Uniprot | |
| Y247 | Phosphorylation | Uniprot | |
| S253 | Phosphorylation | Uniprot | |
| T257 | Phosphorylation | Uniprot | |
| S312 | Phosphorylation | Uniprot | |
| S366 | Phosphorylation | Uniprot | |
| S406 | Phosphorylation | Uniprot | |
| S408 | Phosphorylation | Uniprot | |
| K609 | Sumoylation | Uniprot | |
| K696 | Sumoylation | Uniprot | |
| S711 | Phosphorylation | Uniprot | |
| S739 | Phosphorylation | Uniprot | |
| K745 | Sumoylation | Uniprot | |
| T769 | Phosphorylation | Uniprot | |
| S775 | Phosphorylation | P31749 (AKT1) , O75582 (RPS6KA5) , P17612 (PRKACA) | Uniprot |
| S811 | Phosphorylation | Uniprot |
研究背景
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.
Cytoplasm. Nucleus.
Note: Colocalizes with USP7 in the nucleus.
Widely expressed throughout the body.
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.
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