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-PA006530GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • 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,Rat
  • 免疫原:
    Human DBT
  • 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 0.1% 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
  • 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: 亚洲国产aⅴ综合网| 全部av―极品视觉盛宴| 人妻少妇精品专区性色av| 另类亚洲小说图片综合区| 国产欧美亚洲精品a第一页| 国产午夜福利久久精品 | 亚洲人成亚洲人成在线观看| 西西人体www大胆高清| 寂寞骚妇被后入式爆草抓爆| 免费国产成人高清在线观看网站 | 成人国产精品一区二区免费看| 亚洲人成亚洲人成在线观看 | 真人啪啪高潮喷水呻吟无遮挡| 激情综合一区二区三区| 国产香蕉视频在线播放| 国产成人手机高清在线观看网站 | 东京热人妻中文无码av| 天堂av男人在线播放| 亚洲 综合 欧美在线视频| 久久人人97超碰国产精品| 久久午夜神器| 亚洲日韩精品欧美一区二区一| 国产99久久九九精品无码| 伊人伊成久久人综合网996| 激情按摩系列片aaaa| 日日碰狠狠添天天爽| 中文字字幕在线中文无码| 国产交换配乱婬视频偷网站| 无码播放一区二区三区| 久久久久人妻精品一区蜜桃| 老师黑色丝袜被躁翻了av| 亚洲aⅴ天堂av天堂无码| 国产日韩在线时看高清视频 | 丰满少妇人妻hd高清大乳在线 | 青青草国产线观看| 亚洲人成在线观看网站无码| 国产精品特级毛片一区二区三区| 国产xxxx视频在线| 久久午夜无码鲁丝片直播午夜精品| 亚洲国产欧美在线观看| 国产精品国产成人国产三级|