在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

PQBP1 Antibody

  • 中文名稱:
    PQBP1兔多克隆抗體
  • 貨號:
    CSB-PA018594LA01HU
  • 規格:
    ¥440
  • 圖片:
    • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA018594LA01HU at dilution of 1:100
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) PQBP1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    PQBP1
  • 別名:
    38 kDa nuclear protein containing a WW domain antibody; Mental retardation; X linked 55 antibody; MRX55 antibody; MRXS3 antibody; MRXS8 antibody; Npw38 antibody; Nuclear protein containing WW domain 38 kD antibody; OTTHUMP00000025808 antibody; Polyglutamine binding protein 1 antibody; Polyglutamine tract binding protein 1 antibody; Polyglutamine tract-binding protein 1 antibody; Polyglutamine-binding protein 1 antibody; PQBP 1 antibody; PQBP-1 antibody; PQBP1 antibody; PQBP1_HUMAN antibody; RENS1 antibody; SHS antibody; Sutherland Haan X linked mental retardation syndrome antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Polyglutamine-binding protein 1 protein (2-265AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,PQBP1 Antibody (CSB-PA018594LA01HU),的標記方式是Non-conjugated。對于PQBP1 Antibody,我們還提供其他標記。見下表:

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA018594LB01HU PQBP1 Antibody, HRP conjugated ELISA
    FITC CSB-PA018594LC01HU PQBP1 Antibody, FITC conjugated
    Biotin CSB-PA018594LD01HU PQBP1 Antibody, Biotin conjugated ELISA
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    Intrinsically disordered protein that acts as a scaffold, and which is involved in different processes, such as pre-mRNA splicing, transcription regulation, innate immunity and neuron development. Interacts with splicing-related factors via the intrinsically disordered region and regulates alternative splicing of target pre-mRNA species. May suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. Can activate transcription directly or via association with the transcription machinery. May be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit. Involved in the assembly of cytoplasmic stress granule, possibly by participating in the transport of neuronal RNA granules. Also acts as an innate immune sensor of infection by retroviruses, such as HIV, by detecting the presence of reverse-transcribed DNA in the cytosol. Directly binds retroviral reverse-transcribed DNA in the cytosol and interacts with CGAS, leading to activate the cGAS-STING signaling pathway, triggering type-I interferon production.
  • 基因功能參考文獻:
    1. Results from a study on gene expression variability markers in early-stage human embryos shows that PQBP1 is a putative expression variability marker for the 3-day, 8-cell embryo stage. PMID: 26288249
    2. The WW domain belonging to polyglutamine tract-binding protein 1 (PQBP1) is of particular interest due to its direct involvement in several X chromosome-linked intellectual disabilities, including Golabi-Ito-Hall (GIH) syndrome, where a single point mutation (Y65C) correlates with the development of the disease. The mutant cannot bind to its natural ligand WBP11, which regulates mRNA processing PMID: 27456546
    3. Our data strongly support a gain-of-function pathogenic mechanism of PQBP1 c.459_462delAGAG and c.463_464dupAG mutations, and suggest that therapeutic strategies to restore FMRP function may be beneficial for those patients PMID: 28073926
    4. results suggest that the interaction between PQBP1 and WBP11 negatively modulates the U5-15kD binding of PQBP1 by an allosteric mechanism PMID: 27314904
    5. Study found that PQBP1 directly binds to reverse-transcribed HIV-1 DNA and interacts with cGAS to initiate an IRF3-dependent innate response. PMID: 26046437
    6. Mutations in the PQBP1 gene prevent its interaction with the spliceosomal protein U5-15 kD. PMID: 24781215
    7. The results of this study addressed that the relationship between gene dose and phenotype relationship of dPQBP1 and investigated the mechanism responsible for the lifespan shortening' PMID: 22901698
    8. These data demonstrate a role for PQBP1 in the modulation of stress granules. PMID: 21933836
    9. Data show that the PQBP1 mutation was found in 3 brothers with a phenotype comprising MR, short stature, lean body and microcephaly. PMID: 21315190
    10. Evidence for a functional involvement of the four mutations affecting ATRX (p.1761M4T), PQBP1 (p.155R4X), and SLC6A8 (p.390P4L and p.477S4L), in the etiology of intellectual disability. PMID: 21267006
    11. Whole gene duplication of the PQBP1 gene in syndrome resembling Renpenning. PMID: 21204222
    12. mutations in PQBP1 caused variable loss of cell adhesion from impaired vesicle trafficking disrupts the neuroepithelial lining or neuronal migration and underlies periventricular heterotopia formation PMID: 20886605
    13. frameshift mutations in the PQBP-1 gene lead to expression of mutants lacking the ability to interact with U5-15kD PMID: 20307692
    14. Y65C missense mutation in the WW domain of the Golabi-Ito-Hall syndrome protein PQBP1 affects its binding activity and deregulates pre-mRNA splicing PMID: 20410308
    15. An evaluation of X-linked mental retardation (XLMR) pathology of PQBP1 mutations demonstrated nonsense-mediated mRNA decay and enhance exclusion of the mutant exon. PMID: 19847789
    16. Mutations in the polyglutamine binding protein 1 gene cause X-linked mental retardation PMID: 14634649
    17. mutations cause Renpenning syndrome and X-linked mental retardation with microcephaly PMID: 15024694
    18. Mutations in the polyglutamine-binding protein 1 gene is associated with X-linked mental retardation PMID: 15355434
    19. dysfunction of PQBP-1 induces mitochondrial stress, a key molecular pathomechanism that is shared among human neurodegenerative disorders PMID: 16104847
    20. Pathogenic frameshift mutations in PQBP1 are rare in mentally retarded patients lacking specific associated signs; the 21 bp in-frame deletions may be non-pathogenic, or alternatively could act subtly on PQBP1 function. PMID: 16493439
    21. Data suggest that the SIPP1-PQBP1-induced nuclear inclusions are distinct from the protein aggregates that are associated with polyglutamine diseases and represent dynamic nucleoplasmic heteropolymers of SIPP1 and PQBP1. PMID: 18599155
    22. Results suggest that pqbp-1.1 is involved in lipid metabolism of intestinal cells, and that dysfunction of lipid metabolism might underlie lean body, one of the most frequent symptoms associating with PQBP1-linked mental retrdation patients. PMID: 19119319

    顯示更多

    收起更多

  • 相關疾病:
    Renpenning syndrome 1 (RENS1)
  • 亞細胞定位:
    Nucleus. Nucleus speckle. Cytoplasmic granule.
  • 組織特異性:
    Widely expressed with high level in heart, skeletal muscle, pancreas, spleen, thymus, prostate, ovary, small intestine and peripheral blood leukocytes.
  • 數據庫鏈接:

    HGNC: 9330

    OMIM: 300463

    KEGG: hsa:10084

    STRING: 9606.ENSP00000218224

    UniGene: Hs.534384



主站蜘蛛池模板: 精品久久久久久久无码| 国产av无码专区亚洲草草| 亚洲 小说 欧美 另类 社区| 天堂8在线天堂资源bt| 国产亚洲精品久久久久久无几年桃| 人妻无码中文字幕| 国产在线观看免费视频软件 | 国模无码视频一区二区三区| 日产亚洲一区二区三区| 久久久精品妇女99| 欧美日韩亚洲国产欧美电影| 亚洲熟妇无码av在线播放| 韩国三级hd中文字幕| 亚洲人成无码网www动漫| 欧美人与zoxxxx另类| 人妻体内射精一区二区| 国产熟妇午夜精品aaa| 少妇久久久久久人妻无码| 国产熟人av一二三区| 亚洲精品亚洲人成在线| 无码专区―va亚洲v专区在线| 先锋影音av最新资源网| 黄页网址大全免费观看| 亚洲三区在线观看内射后入| 天天躁日日躁狠狠躁性色av| 国产精品久久久久高潮| 日韩中文字幕在线一区二区三区 | 国产精品真实灌醉女在线播放| 久久久久99精品成人片| 3d动漫精品啪啪一区二区| 国产丝袜在线精品丝袜不卡| 人妻三级日本香港三级极97| 欧洲乱码伦视频免费| 亚洲国产精品不卡av在线 | 国产狂喷潮在线观看中文| 色多多性虎精品无码av| 国产99久9在线视频 | 传媒| 欧美巨大黑人精品videos| 国产成人精品视频一区二区不卡| 欧美极品video粗暴| 亚洲欧美成人中文日韩电影网站|