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

MPV17 Antibody

  • 中文名稱:
    MPV17兔多克隆抗體
  • 貨號(hào):
    CSB-PA014771LA01HU
  • 規(guī)格:
    ¥440
  • 圖片:
    • Western Blot
      Positive WB detected in: U251 whole cell lysate(30μg), SY5Y whole cell lysate(30μg)
      All lanes: MPV17 antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/20000 dilution
      Predicted band size: 20 kDa
      Observed band size: 20 kDa
      Exposure time:120s
    • Western Blot
      Positive WB detected in: Mouse Brain tissue lysate(30μg)
      All lanes: MPV17 antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/20000 dilution
      Predicted band size: 20 kDa
      Observed band size: 20 kDa
      Exposure time: 120s
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品名稱:
    Rabbit anti-Homo sapiens (Human) MPV17 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    MPV17
  • 別名:
    Glomerulosclerosis antibody; Mpv17 antibody; Mpv17 human homolog of glomerulosclerosis and nephrotic syndrome antibody; MpV17 mitochondrial inner membrane protein antibody; MPV17_HUMAN antibody; MTDPS6 antibody; Protein Mpv17 antibody; SYM1 antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Protein Mpv17 protein (1-176AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    Non-conjugated

    本頁面中的產(chǎn)品,MPV17 Antibody (CSB-PA014771LA01HU),的標(biāo)記方式是Non-conjugated。對于MPV17 Antibody,我們還提供其他標(biāo)記。見下表:

    可提供標(biāo)記
    標(biāo)記方式 貨號(hào) 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA014771LB01HU MPV17 Antibody, HRP conjugated ELISA
    FITC CSB-PA014771LC01HU MPV17 Antibody, FITC conjugated
    Biotin CSB-PA014771LD01HU MPV17 Antibody, Biotin conjugated ELISA
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen affinity purification
  • 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(chǎn)品提供形式:
    Liquid
  • 應(yīng)用范圍:
    ELISA, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
  • Protocols:
  • 儲(chǔ)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(chǎn)品評價(jià)

靶點(diǎn)詳情

  • 功能:
    Non-selective channel that modulates the membrane potential under normal conditions and oxidative stress, and is involved in mitochondrial homeostasis. Involved in mitochondrial deoxynucleoside triphosphates (dNTP) pool homeostasis and mitochondrial DNA (mtDNA) maintenance. May be involved in the regulation of reactive oxygen species metabolism and the control of oxidative phosphorylation.
  • 基因功能參考文獻(xiàn):
    1. The authors describe an 11 year old girl, born to consanguineous parents, who presented with rapidly progressive MPV17 hepatocerebral mitochondrial DNA depletion syndrome. Genetic analysis of the patient revealed a homozygous pathogenic mutation c.121C>T (p.R41W) in the MPV17 gene. PMID: 28673863
    2. New case of Navajo Neurohepatopathy presented + literature review is provided to assist in diagnosis and management of the disease. PMID: 28209105
    3. We report a novel homozygous mutation in MPV17 from two unrelated patients harboring axonal sensorimotor polyneuropathy without hepatoencephalopathy. PMID: 26437932
    4. MPV17 is a Deltapsim-modulating channel that apparently contributes to mitochondrial homeostasis under different conditions PMID: 25861990
    5. 12 pathogenic mutations in mitochondrial DNA depletion syndrome in the MPV17 gene, of which 11 are novel, in 17 patients from 12 families. PMID: 23714749
    6. A novel c.191C>G (p.Pro64Arg) MPV17 mutation has been identified in two pairs of unrelated Polish siblings with mitochondrial hepatoencephalopathy. PMID: 23829229
    7. Case Report: functional splicing assay based on the use of minigenes to support that MPV17 c.70 + 5G > A mutation is disease causing. PMID: 20614188
    8. results suggest that M-LPH functions to protect cells from oxidative stress and/or initiation of the mitochondrial apoptotic cascade under stressed conditions PMID: 22306510
    9. eight new patients with seven novel mutations in MPV17 PMID: 20074988
    10. MPV17 is a mitochondrial inner membrane protein, and its absence or malfunction causes oxidative phosphorylation (OXPHOS) failure and mtDNA depletion, not only in affected individuals but also in Mpv17-/- mice PMID: 16582910
    11. These results show the existence of the human homolog of M-LP and its participation in reactive oxygen species metabolism. PMID: 16631601
    12. Sequencing of the MPV17 gene in six patients with Navajo neurohepatopathy from five families revealed the homozygous R50Q mutation described elsewhere. PMID: 16909392
    13. Mutations in the MPV17 gene should be considered in the course of evaluating the molecular etiology for isolated, rapidly progressive infantile hepatic failure. PMID: 17694548
    14. Lack of founder effect for an identical mtDNA depletion syndrome (MDS)-associated MPV17 mutation shared by Navajos and Italians. PMID: 18261905
    15. Lethal hepatopathy, polyneuropathy, neurological regression and leukodystrophy are associated with mutations in MPV17. PMID: 18329934
    16. study describes clinical, molecular morphological & biochemical features of 3 children with hepatocerebral mitochondrial DNA depletion syndrome secondary to novel MPV17 mutations; data confirm MPV17 mutations are associated with a 2-stage syndrome PMID: 18695062
    17. describes in detail the specific clinical and biological characteristics of three patients with MPV17 gene mutations, a rare hepatocerebral mitochondrial DNA depletion syndrome (MDS) PMID: 19012992
    18. clinical courses of patients with MPV17 mutations are greatly influenced by viral infections & dietary & pharmaceutical treatments targeting mitochondrial respiratory chain complex II may be beneficial in the clinical management of MPV17 mutant patients. PMID: 19520594

    顯示更多

    收起更多

  • 相關(guān)疾病:
    Mitochondrial DNA depletion syndrome 6 (MTDPS6)
  • 亞細(xì)胞定位:
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族:
    Peroxisomal membrane protein PXMP2/4 family
  • 組織特異性:
    Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.
  • 數(shù)據(jù)庫鏈接:

    HGNC: 7224

    OMIM: 137960

    KEGG: hsa:4358

    STRING: 9606.ENSP00000233545

    UniGene: Hs.75659



主站蜘蛛池模板: 国产精品欧美亚洲韩国日本久久| 国产mv在线天堂mv免费观看| 日本看片一二三区高清| 国产av精品一区二区三区小说| 欧美巨大xxxx做受中文字幕 | 国内精品免费久久久久软件| 人人玩人人添人人澡欧美| 欧美日韩国产综合新一区| 日日天干夜夜狠狠爱| 国产无遮挡a片又黄又爽| 激情综合色五月六月婷婷| 亚洲欧美人成电影在线观看| 人与嘼av免费| 久久青草费线频观看| 亚洲精品国产嫩草在线观看东京热| 最新69国产成人精品视频| 一区二区三区在线 | 日本| 色一情一乱一伦麻豆| 牲欲强的熟妇农村老妇女视频| 色一情一伦一区二区三| 国产亚洲另类无码专区| 国产精品一区二区 尿失禁| 成人伊人精品色xxxx视频| 大香伊蕉在人线国产网站首页| 性男女做视频观看网站| 欧美日韩亚洲国产在线制服 | 欧洲国产精品无码专区影院| 午夜影视免费| 女人张开腿让男人桶爽| 国产成人无码免费视频97| 浪货趴办公桌~h揉秘书电影| 亚洲人成色777777老人头| 国产精品嫩草影院av| 亚洲精品国产av成拍色拍个| 永久免费无码网站在线观看个| av色综合久久天堂av色综合| 麻豆网神马久久人鬼片| 在线综合亚洲欧美日韩| 在线观看无码av网站永久免费| 日本少妇高潮喷水xxxxxxx| 国产男女无遮挡猛进猛出|