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

Recombinant Human Protein Mpv17 (MPV17)

  • 中文名稱:
    人MPV17重組蛋白
  • 貨號(hào):
    CSB-EP014771HU
  • 規(guī)格:
    ¥1344
  • 圖片:
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他:

產(chǎn)品詳情

  • 純度:
    Greater than 90% as determined by SDS-PAGE.
  • 基因名:
    MPV17
  • Uniprot No.:
  • 別名:
    Glomerulosclerosis; Mpv17; Mpv17 human homolog of glomerulosclerosis and nephrotic syndrome; MpV17 mitochondrial inner membrane protein; MPV17_HUMAN; MTDPS6; Protein Mpv17; SYM1
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長(zhǎng)度:
    Full Length
  • 來(lái)源:
    E.coli
  • 分子量:
    35.7kDa
  • 表達(dá)區(qū)域:
    1-176aa
  • 氨基酸序列
    MALWRAYQRALAAHPWKVQVLTAGSLMGLGDIISQQLVERRGLQEHQRGRTLTMVSLGCGFVGPVVGGWYKVLDRFIPGTTKVDALKKMLLDQGGFAPCFLGCFLPLVGALNGLSAQDNWAKLQRDYPDALITNYYLWPAVQLANFYLVPLHYRLAVVQCVAVIWNSYLSWKAHRL
    Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
    If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
  • 蛋白標(biāo)簽:
    N-terminal 6xHis-SUMO-tagged
  • 產(chǎn)品提供形式:
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 緩沖液:
    If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
    Note: If you have any special requirement for the glycerol content, please remark when you place the order.
    If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
  • 儲(chǔ)存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)期:
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項(xiàng):
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA:
    Please contact us to get it.

產(chǎn)品評(píng)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(kù)鏈接:

    HGNC: 7224

    OMIM: 137960

    KEGG: hsa:4358

    STRING: 9606.ENSP00000233545

    UniGene: Hs.75659



主站蜘蛛池模板: 艳妇乳肉豪妇荡乳av| 蜜臀av 国内精品久久久| 777亚洲精品乱码久久久久久| 亚洲精品国产精品国自产观看| 日韩伦人妻无码| 亚洲爆乳成av人在线蜜芽| 18禁成人黄网站免费观看久久| 两个奶头被吃高潮视频| 国产98在线 | 日韩| 久久精品人人爽人人爽| 18禁黄久久久aaa片| 久久精品中文字幕一区| 西西444www无码大胆| 好男人资源在线www免费| 日产精品一二三区| 日日碰狠狠添天天爽| 日本成熟老妇乱| 国内精品乱码卡一卡2卡| 亚洲欧洲精品成人久久曰影片| 成人性做爰片免费视频| 国产薄丝脚交视频在线观看| 国产精品亚洲mnbav网站| 亚洲精品无码久久| 日韩精品人妻系列无码专区| 四虎影视无码永久免费| 色八区人妻在线视频免费 | 奇米影视7777久久精品| 99国产精品久久久久久久成人 | 成人区人妻精品一区二区不卡视频 | 国语对白刺激精品视频| 亚洲精品一区二三区不卡| 99re6在线视频精品免费| 亚洲精品一区二区三区麻豆| 一边吃奶一边添p好爽高清视频| 国产美女精品一区二区三区| 丝袜无码一区二区三区| 国产成人午夜无码电影在线观看| 天天鲁一鲁摸一摸爽一爽| 99麻豆久久久国产精品免费| 国产1卡2卡3卡4卡免费| 人妻av中文字幕无码专区|