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ATXN7 Antibody

  • 货号：
    CSB-PA002445LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Rat lung tissue
      All lanes: ATXN7 antibody at 3.3μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 96, 102, 80 kDa
      Observed band size: 80 kDa
    • IHC image of CSB-PA002445LA01HU diluted at 1:400 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of A549 cells with CSB-PA002445LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ATXN7 Polyclonal antibody
  • Uniprot No.：
    O15265
  • 基因名：
    ATXN7
  • 别名：
    ATXN7 antibody; SCA7Ataxin-7 antibody; Spinocerebellar ataxia type 7 protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Rat
  • 免疫原：
    Recombinant Human Ataxin-7 protein (402-503AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ATXN7 Antibody (CSB-PA002445LA01HU)，的标记方式是Non-conjugated。对于ATXN7 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA002445LB01HU ATXN7 Antibody, HRP conjugated ELISA
    FITC CSB-PA002445LC01HU ATXN7 Antibody, FITC conjugated
    Biotin CSB-PA002445LD01HU ATXN7 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:300-1:500
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Acts as component of the STAGA transcription coactivator-HAT complex. Mediates the interaction of STAGA complex with the CRX and is involved in CRX-dependent gene activation. Necessary for microtubule cytoskeleton stabilization.
  • 基因功能参考文献：
    1. Genetic testing showed the presence of 48 CAG repeats within one ATXN7 gene for spinocerebellar ataxia type 7 (SCA7). PMID: 28645341
    2. we observed that carriers of either ATXN7 or TBP alleles with relatively large CAG repeat sizes in both alleles had a substantially increased risk of lifetime depression. PMID: 28585930
    3. The intronic SNP rs6798742 is associated with ATXN7 CAG-region expansion. PMID: 28597910
    4. ATXN7 may be a potential predictor of post-operative prognosis of Hepatitis B Virus-related hepatocellular carcinoma . PMID: 27855399
    5. 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
    6. Our study provided the clinico-genetic analysis of nine Indian SCA7 families and CAG repeat distribution analysis in diverse Indian populations showed occurrence of ATXN7-CAG intermediate alleles in a predisposed population PMID: 25900954
    7. Data show that the aggregates formed by polyQ-expanded ataxin 7 sequester ubiquitin-specific protease (USP22) through specific interactions. PMID: 26195632
    8. Two pathological polyglutamine proteins, truncated Ataxin-7 and full-length Ataxin-3, suggest that accumulation of insoluble aggregates beyond a critical threshold could be responsible for neurotoxicity. PMID: 26210447
    9. The proband exhibited a typical phenotype of SCA7, which includes cone dystrophy and spinocerebellar ataxia. PMID: 25643591
    10. Results suggest that sequestration of both enzymatic centers in SAGA upon ATXN7 poly(Q) expansion likely contributes to spinocerebellar ataxia type 7 development and progression. PMID: 25755283
    11. This study shown evidence in vivo, in the SCA7 KI mouse model, that progressive accumulation of mutant ataxin-7 impairs autophagy. PMID: 24859968
    12. Epidemiological evidence of a SCA7 founder effect in a Mexican population with spinocerebellar ataxia. PMID: 23368522
    13. analysis of the founder effect and ancestral origin of the spinocerebellar ataxia type 7 mutation in Mexican families PMID: 24374739
    14. Haplotype and phylogenetic analyses provide evidence showing that the relatively high frequency of SCA7 in Mexican population is the result of a founder mutation and that Mexican SCA7 carriers possess the Western European ancestry. PMID: 23828024
    15. polyQ-expanded ataxin-7 directly bound the Gcn5 catalytic core of SAGA while in association with its regulatory proteins, Ada2 and Ada3. PMID: 24129567
    16. The results demonstrated that a common genetic variant in the ataxia-causing gene ATXN7 influences cerebellar grey matter volume in healthy young adults. PMID: 23100044
    17. Sequestration of the ponsin splice variant R85FL by the polyglutamine-expanded Atx7 in cell is mediated by the specific SH3C-PRR interaction, which is implicated in the pathogenesis of spinocerebellar ataxia 7. PMID: 23892081
    18. role of ataxin-7 in differentiation of photoreceptors and cerebellar neurons PMID: 23226359
    19. Full-length and cleaved fragments of the SCA7 disease protein ataxin-7 (ATXN7) are differentially degraded in a spinocerebellar ataxia type 7 rat model. PMID: 22367614
    20. Critical nuclear events lead to transcriptional alterations in polyglutamine diseases such as spinocerebellar ataxia type 7 (SCA7) and Huntington's disease (HD). PMID: 22917585
    21. The results of this study demonstrated that oxidative stress contributes to ATXN7 aggregation as well as toxicity. PMID: 22827889
    22. ATXN7 distribution frequently shifts from the nucleus to the cytoplasm; cytoplasmic ATXN7 associates with microtubules (MTs); expression of ATXN7 stabilizes MTs; findings provide a novel physiological function of ATXN7 in regulation of cytoskeletal dynamics and suggest that abnormal cytoskeletal regulation may contribute to SCA7 disease pathology PMID: 22100762
    23. The results of this study indicated that SCA7 disease pathogenesis involves a convergence of alterations in a variety of different cell types to fully recapitulate the cerebellar degeneration. PMID: 22072678
    24. The Trinucleotide Repeat Expansion mutation in ATXN7 related to Spinocerebellar ataxia type 7. PMID: 21827908
    25. The interaction between APLP2 and ataxin-7 and proteolytic processing of APLP2 may contribute to the pathogenesis of spinocerebellar ataxia type 7. PMID: 20732423
    26. identified 118 protein interactions for CACNA1A and ATXN7 linking them to other ataxia-causing proteins and the ataxia network; ataxia network is significantly enriched for proteins that interact with known macular degeneration-causing proteins PMID: 21078624
    27. In response to polyglutamine toxicity, transgenic murine SCA7 rods go through a range of radically different cell fates correlating with the nature, level and ratio of mutant transgene ATXN7 species. PMID: 20600911
    28. The solution structures of the SCA7 domain of both ATXN7 and ATXN7L3 reveal a new, common zinc-finger motif at the heart of two distinct folds, providing a molecular basis for the observed functional differences. PMID: 20634802
    29. This study suggested that the SCA7 gene alternation in SCA7 patient in Chinese Han family. PMID: 20739808
    30. These results demonstrate an influence of SUMOylation on the multistep aggregation process of ATXN7 and implicate a role for ATXN7 SUMOylation in SCA7 pathogenesis. PMID: 19843541
    31. analysis of RNA hairpins selective for silencing the mutant ataxin-7 transcript PMID: 19789634
    32. CAG expansion in SCA7 locus is associated with Machado-Joseph disease PMID: 11697524
    33. Activated caspase-3 was recruited into the inclusions in both the cell models and human SCA7 brain and its expression was upregulated in cortical neurones. PMID: 11709544
    34. Expression of ataxin-7 in CNS and non-CNS tissue of normal and SCA7 individuals PMID: 12070661
    35. identification of a novel ataxin-7 protein enriched in the central nervous system suggests that expression of multiple polyglutamine-containing proteins may play a role in the neurodegeneration patterns characteristic of SCA7 PMID: 12533095
    36. Demonstrate here that ataxin-7 is the human orthologue of the yeast SAGA SGF73 subunit and is a bona fide subunit of the human TFTC-like transcriptional complexes. PMID: 15115762
    37. This patient with Spinocerebellar Ataxia 7 due to unique instability of the CAG repeat. PMID: 15316811
    38. Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration in Spinocerebellar ataxia type 7. PMID: 15932940
    39. We show that transcription mediated by both CBP and RORalpha1 was repressed by expanded ataxin-7. Ataxin-7 may act as a repressor of transcription by inhibiting the acetylation activity of TFTC and STAGA. PMID: 15936949
    40. Ataxin-90A aggregates differed morphologically from ataxin7 - 100Q aggregatesand were more toxic to mesencephalic neurons, suggesting that toxicity was determined by the type of aggregate rather than the cellular misfolding response. PMID: 16325416
    41. Trinucleotide repeat expansions of ataxin 7 may be involved in neurodegenerative diseases such as cerebellar ataxia. PMID: 16962040
    42. Origin of the SCA7 gene mutation in South Africa and the possibility of a founder effect in the Black population PMID: 17026624
    43. Massive SCA7 expansion detected in a 7-month-old male with hypotonia, cardiomegaly, and renal failure. PMID: 17254003
    44. We present a pediatric patient with 13 and 70 trinucleotide CAG repeats within SCA7 gene and no family history, whose presentation mimicked Kearns-Sayre syndrome (KSS). PMID: 17720198
    45. expanded CAG-repeats in the SCA7 gene within members of a large Chinese family with spinocerebellar ataxia PMID: 18325672
    46. The patients with genetically confirmed SCA 7 presented an early macular dysfunction, preceding any signs of abnormalities in fundus appearance PMID: 19172503
    47. Ataxin-7 is the human orthologue of SGF73, which is a subunit of the yeast SAGA complex, a coactivator required for transcription of a subset of RNA Pol II-dependent genes. Ataxin-7 is a new subunit of the mammalian SAGA-like complexes, TFTC/STAGA. PMID: 15115762

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  • 相关疾病：
    Spinocerebellar ataxia 7 (SCA7)
  • 亚细胞定位：
    [Isoform a]: Nucleus. Nucleus, nucleolus. Nucleus matrix. Cytoplasm, cytoskeleton. Note=In addition to a diffuse distribution throughout the nucleus, it is associated with the nuclear matrix and the nucleolus. It is able to shuttle between the nucleus and cytoplasm.; [Isoform b]: Cytoplasm.
  • 蛋白家族：
    Ataxin-7 family
  • 组织特异性：
    Isoform a and isoform b are expressed in CNS, but isoform a is expressed predominantly in the peripherical tissues. Isoform b is also highly expressed in the frontal lobe, skeletal muscle and spinal cord and is expressed at a lower level in the lung, lymp
  • 数据库链接：

    HGNC: 10560

    OMIM: 164500

    KEGG: hsa:6314

    STRING: 9606.ENSP00000381590

    UniGene: Hs.476595