ATXN3 Antibody
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中文名稱：ATXN3兔多克隆抗體
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貨號：CSB-PA002443GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：AT3 antibody; Ataxin 3 antibody; ataxin 3 variant h antibody; ataxin 3 variant m antibody; ataxin 3 variant ref antibody; Ataxin-3 antibody; ATX3 antibody; ATX3_HUMAN antibody; ATXN3 antibody; EC 3.4.22. antibody; JOS antibody; Josephin antibody; Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) antibody; Machado Joseph disease antibody; Machado Joseph disease protein 1 antibody; Machado-Joseph disease protein 1 antibody; Machado-Joseph disease protein 1 homolog antibody; MJD antibody; MJD gene antibody; MJD1 antibody; Olivopontocerebellar ataxia 3 antibody; OTTHUMP00000221583 antibody; OTTHUMP00000221585 antibody; OTTHUMP00000221586 antibody; OTTHUMP00000221587 antibody; OTTHUMP00000231995 antibody; OTTHUMP00000231997 antibody; Rsca3 antibody; SCA3 antibody; SCA3 gene antibody; Spinocerebellar ataxia type 3 protein antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human ATX3,ATXN3
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：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. 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.
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基因功能參考文獻：
- Functionally, Ataxin-3 overexpression promoted cell proliferation, and Ataxin-3 knockdown inhibited cell proliferation in testicular cancer cell. In addition, up-regulation of Ataxin-3 inhibited the expression of PTEN and activated the AKT/mTOR pathway. PMID: 29902454
- Low exon skipping efficiencies combined with reduction in important ataxin-3 protein functions suggest that skipping of exon 8 and 9 is not a viable therapeutic option for spinocerebellar ataxia type-3. The modified protein was incapable of binding poly-ubiquitin chains, which may interfere with its normal deubiquitinating function. PMID: 27731380
- Data suggest ATXN3 binds with low-micromolar affinity to both wild-type p97/VCP and mutants linked to proteostasis deficiency multisystem proteinopathy 1 (MSP1; also called hereditary inclusion body myopathy); stoichiometry of binding is one ATXN3 molecule per p97/VCP hexamer in presence of ATP; MSP1 mutants of p97/VCP bind ATXN3 irrespective of nucleotide state. (VCP = valosin-containing protein/ATPase; ATXN3 = ataxin-3) PMID: 28939772
- DNA methylation levels in the ATXN3 promoter were significantly higher in SCA3/MJD patients PMID: 28094059
- data elucidate the important role of ataxin-3 proteolysis in the pathogenesis of Machado-Joseph disease. PMID: 28334907
- The findings reveal ATXN3 to be a novel deubiquitinase of Chk1, providing a new mechanism of Chk1 stabilization in genome integrity maintenance. PMID: 28180282
- Segregation patterns and factors influencing instability of expanded ATXN3 CAG transmissions in Machado-Joseph disease have been analyzed. PMID: 26693702
- Our data reveal a previously unrecognized balance between pathogenic and potentially therapeutic properties of the ataxin-3-Rad23 interaction; they highlight this interaction as critical for the toxicity of the SCA3 protein, and emphasize the importance of considering protein context when pursuing suppressive avenues. PMID: 28158474
- the opposing activities of RNF4 and ataxin-3 consolidate robust MDC1-dependent signaling and repair ofDNA double-strand break. PMID: 28275011
- we demonstrated that neural differentiation in these iPS cells was accompanied by autophagy and that rapamycin promoted autophagy through degradation of mutant ATXN3 proteins in neurally differentiated spinocerebellar ataxia-3 human induced pluripotent stem cells (p < 0.05). In conclusion, patient-derived iPS cells are a good model for studying the mechanisms of SCA3 and may provide a tool for drug discovery in vitro. PMID: 27847820
- South American cohort did not confirm the effect of the four candidate loci as modifier of onset age: mithocondrial A10398G polymorphism and CAGn at RAI1, CACNA1A, ATXN3, and ATXN7 genes PMID: 25869926
- Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models. PMID: 26880203
- USP19_b up-regulates the protein levels of the polyglutamine (polyQ)-containing proteins, ataxin-3 (Atx3) and huntingtin (Htt), and thus promotes aggregation of their polyQ-expanded species in cell models PMID: 26808260
- Based on these data and other related studies, we presumed that de novo mutations of ATXN3 emerging from large ANs are at least one survival mechanisms of mutational ATXN3 and we can redefine the range of CAG repeats as: ANs/=50 PMID: 26266536
- Results suggest that the aggregation of Josephin proceeds from the monomer state to the formation of spheroidal intermediates with a native structure. Only successively, these intermediates evolve into misfolded aggregates and into the final fibrils. PMID: 26215704
- This study did not find relationship between CAG expansion length and psychiatric disorders. PMID: 26067219
- Data show that homozygosity for Machado-Joseph disease (MJD)/SCA3 protein enhances the clinical severity of the disease. PMID: 25566755
- A multistage aggregation mechanism for ataxin-3 is described in which flanking domain self-assembly precedes polyglutamine aggregation yet is influenced by polyglutamine expansion. PMID: 25700012
- Ubiquitination of ataxin-3 is not necessary for its proteasomal degradation.Ataxin-3 is regulated by ubiquitin-binding site 2 on its N terminus.Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23. PMID: 25144244
- Machado-Joseph disease patients carrying the rs709930 A allele and rs910369 T allele of ATXN3 experienced an earlier age of onset of approximately 2 to 4 years. PMID: 25689313
- polyglutamine expansion increases the molecular mobility of two juxtaposed helices critical to ataxin-3 deubiquitinase activity PMID: 26260925
- Evaluated the level of truncated pathological recombinant Ataxin-3 in a Drosophila model, in presence or absence of two suppressors and during aging. Suppressing truncated Ataxin-3-induced toxicity lowered the level of aggregated polyglutamine protein. PMID: 26210447
- Data support the importance of ATXN3 in neuronal cells and indicate that an expanded polyQ tract leads to a partial loss of the cellular function of ATXN3 that may be relevant to neurodegeneration. PMID: 25143392
- Wide type and polyQ-expanded ataxin-3 both showed partial co-localization to endoplasmic reticulum. PMID: 26037349
- Here we report that purified wild-type (WT) ATXN3 stimulates, and by contrast the mutant form specifically inhibits, PNKP's 3' phosphatase activity in vitro. ATXN3-deficient cells also show decreased PNKP activity PMID: 25633985
- We now report that the mutant ATXN3 protein interacts with and inactivates PNKP (polynucleotide kinase 3'-phosphatase), an essential DNA strand break repair enzyme PMID: 25590633
- The At3 N-terminal Josephin domain aggregation might be a multistep process. PMID: 25268243
- Results suggest that polyglutamine-expanded ataxin-3-Q79 impairs histone acetyltransferase activity, leading to impaired induction of cerebellar long-term depression in the spinocerebellar ataxia type 3 transgenic mouse PMID: 25139423
- Authors have investigated the interaction of AT3 with tubulin and HDAC6. PMID: 24685680
- miR-25 reduced both wild-type and polyQ-expanded mutant ataxin-3 protein levels by interacting with the 3'UTR of ATXN3 mRNA PMID: 25451224
- substrate recognition by the Josephin domain of ataxin-3 PMID: 25448680
- ataxin-3 fragment aggregates in a polyQ length-dependent manner in C. elegans muscle cells and that this aggregation is associated with cellular dysfunction PMID: 24817148
- In ATXN3-depleted cells, under conditions of transcriptional inhibition, PTEN and PTENP1 mRNAs rapidly decay. PMID: 24292675
- interaction between UbD2 and p97/Atx3 mediates retranslocation of UbD2 to the cytoplasm for terminal degradation in the proteasomes. PMID: 24196352
- Our work also suggests that ataxin-3 suppresses degeneration by regulating toxic protein aggregation rather than stability. PMID: 24106274
- The cloned A3IP gene encodes A3IP, a novel ataxin-3 interacting protein. PMID: 23926002
- Our data suggest that ataxin-3 plays an important role in regulating the Bcl-XL-Bax-mediated anti-oxidative response by modulating the interaction between Bcl-XL and Bax. PMID: 23562578
- This study demonistrated that Altered expression of carbonic anhydrase-related protein XI in neuronal cells expressing mutant ataxin-3. PMID: 23184527
- our study demonstrated that SUMOylation on K166, the first described residue of SUMO-1 modification of ataxin-3, partially increased the stability of mutant-type ataxin-3, and the rate of apoptosis arisen from the cytotoxicity of the modified protein PMID: 23382880
- 10 SNPs (none in core splicing signals) were found from exonic & flanking intronic regions in genomic DNA from Machado-Joseph disease patients & controls. The SNPs implied losses & gains of splicing-factor-recognition motifs. PMID: 22706685
- the efficacy of gene silencing in blocking the MJD-associated motor-behavior and neuropathological abnormalities PMID: 23349684
- Ataxin-3 is cleaved by calpains. Increased proteolytic cleavage of ataxin-3 results in a more severe and faster progressing neurological phenotype of spinocerebellar ataxia type 3. PMID: 23100324
- VCP/p97 was shown to be an activator specifically of wild-type ataxin-3. PMID: 22970133
- no functional effect could be predicted for ATXN3 gene variant. PMID: 22422287
- the sequestration of misfolded SOD1 into aggresomes, which is driven by ataxin-3, plays an important role in attenuating protein misfolding-induced cell toxicity. PMID: 22761419
- ATX3 proteolysis in transgenic mice by human calpains mediates ATX3 translocation to the nucleus, aggregation and toxicity. PMID: 22843411
- Transiently transfected HEK cell lines with expanded (Q84) ataxin-3 exhibited a higher susceptibility to 3-nitropropionic acid (3-NP), an irreversible inhibitor of mitochondrial complex II. PMID: 22037589
- These results underscore ataxin 3 capability of undergoing multiple aggregation pathways that lead to end products endowed with substantially different molecular structures. PMID: 22234302
- This study demonistrated that Disease progressed of autosomal dominant cerebellar ataxia and spastic paraplegia faster in SCA s with polyglutamine expansions in SCA1, 2, and 3. PMID: 22491195
- the central flexible region enhances protein aggregation and can populate conformational states with different degrees of compactness PMID: 22129356
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相關疾病：Spinocerebellar ataxia 3 (SCA3)
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亞細胞定位：Nucleus matrix. Nucleus. Note=Predominantly nuclear, but not exclusively, inner nuclear matrix.
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組織特異性：Ubiquitous.
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數據庫鏈接：
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