产品描述

来源:
Mouse
应用:
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.
*Tips:

WB: 适用于变性蛋白样本的免疫印迹检测. IHC: 适用于组织样本的石蜡(IHC-p)或冰冻(IHC-f)切片样本的免疫组化/荧光检测. IF/ICC: 适用于细胞样本的荧光检测. ELISA(peptide): 适用于抗原肽的ELISA检测.

反应:
Human
克隆:
Monoclonal [AFB1848]
特异性:
ATXN1 antibody detects endogenous levels of total ATXN1.
RRID:
AB_2833872
引用格式: Affinity Biosciences Cat# BF0249, RRID:AB_2833872.
偶联:
Unconjugated.
纯化:
Affinity-chromatography.
保存:
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.
别名:

展开/折叠

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

抗原和靶标

免疫原:

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

Uniprot:
基因/基因ID:
表达:
P54253 ATX1_HUMAN:

Widely expressed throughout the body.

描述:
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.
序列:
MKSNQERSNECLPPKKREIPATSRSSEEKAPTLPSDNHRVEGTAWLPGNPGGRGHGGGRHGPAGTSVELGLQQGIGLHKALSTGLDYSPPSAPRSVPVATTLPAAYATPQPGTPVSPVQYAHLPHTFQFIGSSQYSGTYASFIPSQLIPPTANPVTSAVASAAGATTPSQRSQLEAYSTLLANMGSLSQTPGHKAEQQQQQQQQQQQQHQHQQQQQQQQQQQQQQHLSRAPGLITPGSPPPAQQNQYVHISSSPQNTGRTASPPAIPVHLHPHQTMIPHTLTLGPPSQVVMQYADSGSHFVPREATKKAESSRLQQAIQAKEVLNGEMEKSRRYGAPSSADLGLGKAGGKSVPHPYESRHVVVHPSPSDYSSRDPSGVRASVMVLPNSNTPAADLEVQQATHREASPSTLNDKSGLHLGKPGHRSYALSPHTVIQTTHSASEPLPVGLPATAFYAGTQPPVIGYLSGQQQAITYAGSLPQHLVIPGTQPLLIPVGSTDMEASGAAPAIVTSSPQFAAVPHTFVTTALPKSENFNPEALVTQAAYPAMVQAQIHLPVVQSVASPAAAPPTLPPYFMKGSIIQLANGELKKVEDLKTEDFIQSAEISNDLKIDSSTVERIEDSHSPGVAVIQFAVGEHRAQVSVEVLVEYPFFVFGQGWSSCCPERTSQLFDLPCSKLSVGDVCISLTLKNLKNGSVKKGQPVDPASVLLKHSKADGLAGSRHRYAEQENGINQGSAQMLSENGELKFPEKMGLPAAPFLTKIEPSKPAATRKRRWSAPESRKLEKSEDEPPLTLPKPSLIPQEVKICIEGRSNVGK

翻译修饰 - P54253 作为底物

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

研究背景

功能:

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).

翻译修饰:

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.

细胞定位:

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
组织特异性:

Widely expressed throughout the body.

亚基结构:

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).

蛋白家族:

The AXH domain is required for interaction with CIC.

Belongs to the ATXN1 family.

限制条款

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