Ataxin 1 Recombinant Rabbit mAb - #BF3811

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製品: Ataxin 1 Recombinant Rabbit mAb
カタログ: BF3811
タンパク質の説明: Rabbit monoclonal antibody to Ataxin 1
アプリケーション: WB
反応性: Human, Mouse
分子量: 105 kDa(Observed); 87kD(Calculated).
ユニプロット: P54253

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製品説明

ソース:
Rabbit IgG
アプリケーション:
WB 1:1000-1:3000
*The optimal dilutions should be determined by the end user. For optimal experimental results, antibody reuse is not recommended.
*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.

反応性:
Human, Mouse
クローナリティ:
Monoclonal [ReFirm23258]
特異性:
Ataxin 1 Recombinant Rabbit mAb detects endogenous levels of Ataxin 1.
コンジュゲート:
Unconjugated.
精製:
Affinity-chromatography.
保存:
Rabbit IgG in Tris-Glycine (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.
別名:

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

免疫原:

A synthetic peptide from human Ataxin 1

Uniprot:

P54253(ATX1_HUMAN) >>Visit HPA database.

遺伝子(ID):
ATXN1(6310)
発現特異性:
P54253 ATX1_HUMAN:

Widely expressed throughout the body.

タンパク質の説明:
The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum,brain stem and spinal cord. Clinically,ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous,with five genetic loci,designated spinocerebellar ataxia (SCA) 1,2,3,4 and 6,being assigned to five different chromosomes. ADCAII,which always presents with retinal degeneration (SCA7),and ADCAIII often referred to as the `pure' cerebellar syndrome (SCA5),are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats,producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable,usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6,and it has been determined that the diseased allele contains 40-83 CAG repeats,compared to 6-39 in the normal allele,and is associated with spinocerebellar ataxia type 1 (SCA1). At least two transcript variants encoding the same protein have been found for this gene.
タンパク質配列:
MKSNQERSNECLPPKKREIPATSRSSEEKAPTLPSDNHRVEGTAWLPGNPGGRGHGGGRHGPAGTSVELGLQQGIGLHKALSTGLDYSPPSAPRSVPVATTLPAAYATPQPGTPVSPVQYAHLPHTFQFIGSSQYSGTYASFIPSQLIPPTANPVTSAVASAAGATTPSQRSQLEAYSTLLANMGSLSQTPGHKAEQQQQQQQQQQQQHQHQQQQQQQQQQQQQQHLSRAPGLITPGSPPPAQQNQYVHISSSPQNTGRTASPPAIPVHLHPHQTMIPHTLTLGPPSQVVMQYADSGSHFVPREATKKAESSRLQQAIQAKEVLNGEMEKSRRYGAPSSADLGLGKAGGKSVPHPYESRHVVVHPSPSDYSSRDPSGVRASVMVLPNSNTPAADLEVQQATHREASPSTLNDKSGLHLGKPGHRSYALSPHTVIQTTHSASEPLPVGLPATAFYAGTQPPVIGYLSGQQQAITYAGSLPQHLVIPGTQPLLIPVGSTDMEASGAAPAIVTSSPQFAAVPHTFVTTALPKSENFNPEALVTQAAYPAMVQAQIHLPVVQSVASPAAAPPTLPPYFMKGSIIQLANGELKKVEDLKTEDFIQSAEISNDLKIDSSTVERIEDSHSPGVAVIQFAVGEHRAQVSVEVLVEYPFFVFGQGWSSCCPERTSQLFDLPCSKLSVGDVCISLTLKNLKNGSVKKGQPVDPASVLLKHSKADGLAGSRHRYAEQENGINQGSAQMLSENGELKFPEKMGLPAAPFLTKIEPSKPAATRKRRWSAPESRKLEKSEDEPPLTLPKPSLIPQEVKICIEGRSNVGK

研究背景

機能:

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).

PTMs:

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.

細胞の位置付け:

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
組織特異性:

Widely expressed throughout the body.

タンパク質ファミリー:

The AXH domain is required for interaction with CIC.

Belongs to the ATXN1 family.

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