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

DBT Antibody

  • 中文名稱:
    DBT兔多克隆抗體
  • 貨號:
    CSB-PA006530LA01HU
  • 規格:
    ¥440
  • 圖片:
    • Western Blot
      Positive WB detected in: 293 whole cell lysate, Mouse kidney tissue, Mouse liver tissue
      All lanes: DBT antibody at 4.3µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 54 kDa
      Observed band size: 54 kDa
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) DBT Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    DBT
  • 別名:
    DBT antibody; BCATE2 antibody; Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex antibody; mitochondrial antibody; EC 2.3.1.168 antibody; 52 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody; Branched chain 2-oxo-acid dehydrogenase complex component E2 antibody; BCOADC-E2 antibody; Branched-chain alpha-keto acid dehydrogenase complex component E2 antibody; BCKAD-E2 antibody; BCKADE2 antibody; Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex antibody; Dihydrolipoamide branched chain transacylase antibody; Dihydrolipoyllysine-residue antibody; 2-methylpropanoyl)transferase antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial protein (140-259AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,DBT Antibody (CSB-PA006530LA01HU),的標記方式是Non-conjugated。對于DBT 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-PA006530LB01HU DBT Antibody, HRP conjugated ELISA
    FITC CSB-PA006530LC01HU DBT Antibody, FITC conjugated
    Biotin CSB-PA006530LD01HU DBT 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, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:5000
  • 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:
    The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.
  • 基因功能參考文獻:
    1. The novel DBT mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion indicating a possible founder mutation may be preserved. PMID: 24268812
    2. Deletion in DBT gene is associated with maple syrup urine disease. PMID: 23313820
    3. 4 novel mutations in DBT gene resulting in intermittent maple syrup urine disease in 7 Norwegian patients; pathogenic effect of the mutations is depletion of cellular protein; intermittent form of MSUD appears to be due to residual R301C mutant protein PMID: 20570198
    4. a distinct subset of antimitochondrial antibodies recognize sequences on branched-chain acyltransferase which located outside of the lipoyl binding domain, in primary biliary cirrhosis and overlap syndrome with autoimmune hepatitis PMID: 14768949
    5. presence of the interdomain linker restricts the motional freedom of the hbSBD more significantly than hbLBD, and that the linker region likely exists as a soft rod rather than a flexible string in solution. PMID: 16861235
    6. in our cohort more severe enzyme & clinical phenotypes of variant maple syrup urine disease were mainly associated with specific genotypes in BCKDHA gene; milder enzyme & clinical phenotypes were associated with specific genotypes in BCKDHB & DBT genes PMID: 17922217
    7. 30 Maple syrup urine disease Portuguese patients studied; 17 putative mutations have been identified (6 in BCKDHA, 5 in BCKDHB and 6 in DBT); 7 of are described for the first time. PMID: 18378174
    8. Examination of the deletion mutation in the E2 (DBT) gene facilitated early MSUD diagnosis and was beneficial for the determination of the proper course of treatment. PMID: 18533943
    9. In 37% (12 patients) of a total of 64 alleles, the supposed maple syrup urine disease-causing mutations in Turkish patients were located in the BCKDHA gene, in 44% (14 patients) in the BCKDHB gene and in 19% (6 patients) in the DBT gene. PMID: 19480318
    10. two novel type IB MSUD mutations in Israeli patients, which affect the E1beta subunit in the decarboxylase (E1) component of the branched-chain alpha-ketoacid dehydrogenase complex PMID: 11448970
    11. Mutation in DBT causes a subset of maple syrup urine disease in Ashkenazi Jewish population. PMID: 11509994

    顯示更多

    收起更多

  • 相關疾病:
    Maple syrup urine disease 2 (MSUD2)
  • 亞細胞定位:
    Mitochondrion matrix.
  • 蛋白家族:
    2-oxoacid dehydrogenase family
  • 數據庫鏈接:

    HGNC: 2698

    OMIM: 248600

    KEGG: hsa:1629

    STRING: 9606.ENSP00000359151

    UniGene: Hs.709187



主站蜘蛛池模板: 亚洲a∨国产av综合av下载| 乱码人妻一区二区三区| 国产亚洲精品久久久久小| 亚洲熟女乱综合一区二区在线 | 伊人色综合网一区二区三区| 国产国产成人久久精品| 天天爽夜夜爽视频精品| 国产精品久久久久久久久久免费看| 精品深夜av无码一区二区| 国产性猛交普通话对白| 久久久久青草线蕉亚洲麻豆| 亚洲精品人成网线在播放va| 欧美大片aaaaa免费观看| 丁香激情综合久久伊人久久| 中文www新版资源在线| 青青久在线视观看视| 色五月丁香五月综合五月| 国产欧美日韩va另类在线播放| 国产毛片欧美毛片久久久| 少妇厨房愉情理伦bd在线观看| 亚洲国产99精品国自产拍| 在线无码av一区二区三区| 亚洲第一无码xxxxxx| 99热这里有精品| 国产女人高潮嗷嗷嗷叫| 少妇被又大又粗猛烈进出视频| 亚洲人成色77777在线观看大战p| 风韵少妇性饥渴推油按摩视频 | 午夜成人爽爽爽视频在线观看| 国产一区二区亚洲精品| 被黑人猛躁10次高潮视频| 国产人妻人伦精品1国产丝袜| 亚洲中文字幕婷婷在线| 精品日产一卡二卡| 欧美野性肉体狂欢大派对| 成人永久免费网站在线观看 | 免费人成网站在线观看欧美| 无码伊人66久久大杳蕉网站谷歌 | 欧美裸体xxxx极品少妇| 国产aⅴ精品一区二区三区尤物| 男女做爰无遮挡性视频|