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 Mitochondrial ornithine transporter 1 (SLC25A15)

In Stock
  • 中文名稱:
    人SLC25A15重組蛋白
  • 貨號:
    CSB-EP897578HU
  • 規格:
    ¥1536
  • 圖片:
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他:

產品詳情

  • 純度:
    Greater than 90% as determined by SDS-PAGE.
  • 基因名:
    SLC25A15
  • Uniprot No.:
  • 別名:
    HHH; Mitochondrial ornithine transporter 1; ORC1; Ornithine transporter 1; Ornithine transporter, mitochondrial; ORNT1; ORNT1_HUMAN; SLC25A15; Solute carrier family 25 (Mitochondrial carrier, ornithine transporter) member 15; Solute carrier family 25 member 15
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Full Length
  • 來源:
    E.coli
  • 分子量:
    59.7kDa
  • 表達區域:
    1-301aa
  • 氨基酸序列
    MKSNPAIQAAIDLTAGAAGGTACVLTGQPFDTMKVKMQTFPDLYRGLTDCCLKTYSQVGFRGFYKGTSPALIANIAENSVLFMCYGFCQQVVRKVAGLDKQAKLSDLQNAAAGSFASAFAALVLCPTELVKCRLQTMYEMETSGKIAKSQNTVWSVIKSILRKDGPLGFYHGLSSTLLREVPGYFFFFGGYELSRSFFASGRSKDELGPVPLMLSGGVGGICLWLAVYPVDCIKSRIQVLSMSGKQAGFIRTFINVVKNEGITALYSGLKPTMIRAFPANGALFLAYEYSRKLMMNQLEAY
    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.
  • 蛋白標簽:
    N-terminal GST-tagged
  • 產品提供形式:
    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.
  • 儲存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期:
    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:
    3-7 business days
  • 注意事項:
    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.

產品評價

靶點詳情

  • 功能:
    Ornithine-citrulline antiporter. Connects the cytosolic and the intramitochondrial reactions of the urea cycle by exchanging cytosolic ornithine with matrix citrulline. The stoichiometry is close to 1:1.
  • 基因功能參考文獻:
    1. characterized mutations of the proposed substrate binding site in ORC1 and ORC2; demonstrated that the residue at position 179 in the 2 soforms is largely responsible for the difference in their substrate specificity;concluded that Arg-179 is a key residue in the opening of the carrier to the matrix side PMID: 22262851
    2. Mutation analysis revealed two novel mutations in the ORNT1 gene. PMID: 22465082
    3. Useful insights for in-depth understanding of the molecular mechanism of the HHH syndrome and developing effective drugs against the disease. PMID: 22292090
    4. novel mutations in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome PMID: 11668643
    5. expression, reconstitution, functional characterization, and tissue distribution of two human isoforms PMID: 12807890
    6. Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family. PMID: 14759633
    7. The DeltaF 188 mutant was not incorporated into the membrane to the same extent as wild type, but retained significant residual activity and lost stereospecificity. PMID: 16256388
    8. A novel R275X mutation of the SLC25A15 gene in a Japanese patient with the HHH syndrome PMID: 16376511
    9. The three patients were homozygous for a novel mutation in ORNT1 with a Gly220Arg change. We suggest including HHH syndrome in the differential diagnosis of patients found to have stroke-like lesions on brain MRI. PMID: 17825324
    10. Clinical presentations and outcomes varied significantly in HHH syndrome patients homozygous for delF188 mutations in SLC25A15. PMID: 18978333
    11. 16 additional Hyperornithinemia-hyperammonemia-homocitrullinuria cases were collected and the spectrum of SLC25A15/ORC1 mutations, was expanded. PMID: 19242930

    顯示更多

    收起更多

  • 相關疾病:
    Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH syndrome)
  • 亞細胞定位:
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族:
    Mitochondrial carrier (TC 2.A.29) family
  • 組織特異性:
    Highly expressed in liver, pancreas, testis, lung and small intestine. Lower levels are detected in spleen, kidney, brain and heart.
  • 數據庫鏈接:

    HGNC: 10985

    OMIM: 238970

    KEGG: hsa:10166

    STRING: 9606.ENSP00000342267

    UniGene: Hs.646645



主站蜘蛛池模板: 狠狠色噜噜狠狠狠狠888奇禾| 久久国产乱子伦免费精品| 国产极品粉嫩泬免费观看| 国产色在线 | 亚洲| 亚洲情xo亚洲色xo无码| 久久中文字幕无码中文字幕有码 | 国产成a人亚洲精品无码久久网| 青草内射中出高潮| 韩国无码av片在线观看| 国产色情又大又粗又黄的电影| 国产成人无码一二三区视频| 在线观看无码不卡av| 色综合欧美亚洲国产| 小污女导航福利入口| 无码丰满熟妇juliaann与黑人| 免费大黄网站| 人妻丰满熟妇av无码片| 亚洲 制服 丝袜 无码 在线| 成人区人妻精品一区二区三区 | 精品无码久久久久久国产| 欧美色欧美亚洲日韩在线播放 | 亚洲精品成人网站在线播放| 国产高清色高清在线观看| 日本japanese丰满少妇| 日本三级手机在线播放线观看| 在线人成免费视频69国产 | 狠狠cao日日穞夜夜穞av| 麻豆文化传媒精品一区观看| 精品亚洲成a人片在线观看 | 国产精品96久久久久久| 日本毛茸茸的丰满熟妇| 中文字幕久久久久人妻| 国产伦精品免编号公布| 国产喷水福利在线视频| 亚洲国产成人久久综合同性| 曰批全过程免费视频在线观看无码| 日本高清aⅴ毛片免费| 丝袜美腿丝袜亚洲综合| 东京热大乱系列无码| 少妇高潮毛片免费看| 男女真人国产牲交a做片野外|