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

PRPS1 Antibody

  • 中文名稱:
    PRPS1兔多克隆抗體
  • 貨號:
    CSB-PA018776GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    PRPS1
  • 別名:
    ARTS antibody; CMTX5 antibody; Deafness 2 perceptive congenital antibody; Deafness X linked 2 perceptive congenital antibody; DFN2 antibody; DFNX1 antibody; EC 2.7.6.1 antibody; KIAA0967 antibody; Phosphoribosyl pyrophosphate synthase I antibody; Phosphoribosyl pyrophosphate synthetase I antibody; PPRibP antibody; Prps1 antibody; PRPS1_HUMAN antibody; PRS I antibody; PRS-I antibody; PRSI antibody; Ribose phosphate pyrophosphokinase I antibody; Ribose-phosphate pyrophosphokinase 1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human PRPS1
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB,IHC,IF
  • 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:
    Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
  • 基因功能參考文獻:
    1. suggests that the four mutations might affect the PRPS1 protein function and stability of the structure PMID: 29047041
    2. This report highlights the unexpected finding of retinal degeneration in females caused by missense variants in the X-linked gene PRPS1 and expands our understanding of the phenotypic outcome of specific variants. PMID: 28967191
    3. ARTS binds directly to both XIAP and Bcl-2. ARTS functions as a distinct Bcl-2 antagonist by binding and leading to its degradation. PMID: 29020630
    4. A novel PRPS1 mutation related to early-onset progressive nonsyndromic hearing loss. PMID: 27886419
    5. High PRPS1 expression is associated with chemoresistance in breast cancer. PMID: 28177767
    6. evaluation of current literature on PRPS1-related syndromes and summaries of potential therapies [review] PMID: 26089585
    7. analysis of intrafamilial phenotypic variation associated with a single PRPS1 mutation in syndromic or nonsyndromic hearing impairment PMID: 25785835
    8. CRC cells that overexpressed miR124 or with knockdown of RPIA or PRPS1 had reduced DNA synthesis and proliferation, whereas cells incubated with an inhibitor of miR124 had significantly increased DNA synthesis and proliferation and formed more colonies. PMID: 26248089
    9. Study identified the critical region in the ARTS promoter and demonstrated that the Sp1 transcription factor could regulate the activity of the ARTS promoter through multiple Sp1 binding sites. PMID: 25790304
    10. Females with a missense mutation in PRPS1, exhibit neuropathy, hearing loss and retinopathy. PMID: 25491489
    11. the de novo purine synthesis inhibitor lometrexol effectively abrogated PRPS1 mutant-driven drug resistance. PMID: 25962120
    12. The expression of different genes encoding subunits of PRPS enzyme is affected by hypoxia in tumor glioma cells, but the effect of hypoxia is modified by suppression of endoplasmic reticulum stress signaling enzyme ERN1. PMID: 25816608
    13. respective phenotypic presentation seems to be determined by the exact PRPS1 mutation and the residual enzyme activity, the latter being largely influenced by the degree of skewed X-inactivation PMID: 24528855
    14. The crystal structure of the ADP-binding pocket of the PRPS1 D52H-mutant and evidence of reduced inhibitor sensitivity. PMID: 23509005
    15. Review: discuss role of PRPS1 mutations in hearing loss. PMID: 23190330
    16. missense mutations in PRPS1 can cause a continuous spectrum of features ranging from progressive non-syndromic postlingual hearing impairment to uric acid overproduction, and recurrent infections depending on the functional sites that are affected PMID: 22246954
    17. Translocation of ARTS initiates a first wave of caspase activation leading to the subsequent release of additional mitochondrial factors, including cytochrome C and SMAC/Diablo. PMID: 21869827
    18. Three HPRT1 mutations in Lesch-Nyhan families were identified but no mutation was identified in any patient in the analysis of PRPS1. PMID: 22132986
    19. Refinements were made on the DFN2 locus on the X chromosome. PMID: 15240907
    20. In four hyperuricemic patients with mild neurological abnormality, molecular analysis of PRPS1 was performed , but no mutations in PRPP synthetase were found. PMID: 20544509
    21. PRPS1 mutations; neurological phenotype in all four PRPS1-related disorders seems to result primarily from reduced levels of GTP & possibly other purine nucleotides including ATP, suggesting these disorders belong to the same disease spectrum [review] PMID: 20380929
    22. PRPS1 loss of function mutations cause a type of nonsyndromic X-linked sensorineural deafness, DFN2 PMID: 20021999
    23. p300 may play a role in the regulation of DNA synthesis through interactions with PRS1 PMID: 15943588
    24. PRS1 belongs to space group R3, with unit-cell parameters a=b=168.846, c=61.857 A, assuming two molecules in asymmetric unit & volume-to-weight ratio of 2.4 angstroms3 Da(-1), which was consistent with result calculated from self-rotation function. PMID: 16682768
    25. new SO4(2-)-binding site is a second allosteric site to regulate the enzymatic activity PMID: 16939420
    26. Linkage analysis in a Dutch family and an Australian family suggested that the candidate gene for Arts syndrome is PRPS1, which maps to Xq22.1-q24. PMID: 17701896
    27. Missense mutations were identified at conserved amino acids in the PRPS1 gene on Xq22.3 in two families with a syndromic form of inherited peripheral neuropathy, one of Asian and one of European descent. PMID: 17701900
    28. increased activity of this gene leads to gout PMID: 18409517
    29. The N114S mutation alters the secondary structure of PRS1;the structural alteration caused by the N114S mutation influences the conformation of the ATP-binding loop and leads to the loss of ATP-induced aggregation. PMID: 19161981

    顯示更多

    收起更多

  • 相關疾?。?/div>
    Phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity); Charcot-Marie-Tooth disease, X-linked recessive, 5 (CMTX5); ARTS syndrome (ARTS); Deafness, X-linked, 1 (DFNX1)
  • 蛋白家族:
    Ribose-phosphate pyrophosphokinase family
  • 數據庫鏈接:

    HGNC: 9462

    OMIM: 300661

    KEGG: hsa:5631

    STRING: 9606.ENSP00000361512

    UniGene: Hs.56



主站蜘蛛池模板: 久久久免费精品re6| 极品人妻videosss人妻| 中国亚洲女人69内射少妇| 国产亚洲精久久久久久无码77777| 午夜好爽好舒服免费视频| 精品免费国偷自产在线视频| 天干夜天干天天天爽视频 | 2019nv天堂香蕉在线观看| 中文字幕无码毛片免费看| 国产在线第一区二区三区| 欧美亚洲日本高清不卡| 国产看黄网站又黄又爽又色| 成人看片黄a免费看在线| 18禁黄污无遮挡无码网站| 人妻少妇久久中文字幕| 天天综合天天做天天综合| 免费国产午夜理论片不卡| 国产精品免费视频色拍拍| 66亚洲一卡2卡新区成片发布| 国产 一二三四五六| 狼友av永久网站免费观看孕交| 国产成人不卡无码免费视频| 97色偷偷色噜噜狠狠爱网站| 亚洲人成无码网www电影麻豆 | 极品美女在线观看免费直播| 国产亚洲福利在线视频| 忘忧草影院在线www韩国日本| 宅女午夜福利免费视频| 国产永久免费观看视频| 亚洲 日韩 欧美 成人 在线| 亚洲欧美黑人深喉猛交群| 国产精品免费精品自在线观看| 一线二线无人区乱码| 国内精品2020情侣视频| 国产成人一区二区三区| 成人亚洲精品久久久久| 丰满的少妇邻居中文bd| 伴郎粗大的内捧猛烈进出视频观看| 凹凸精品熟女在线观看| 国产成久久免费精品av片| 成人性生交大免费看|