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

ACAD9 Antibody

  • 中文名稱:
    ACAD9兔多克隆抗體
  • 貨號:
    CSB-PA887999EA01HU
  • 規格:
    ¥440
  • 圖片:
    • Western Blot
      Positive WB detected in: MCF-7 whole cell lysate, HEK293 whole cell lysate, K562 whole cell lysate, Mouse heart tissue, Mouse brain tissue, Mouse liver tissue
      All lanes: ACAD9 antibody at 1μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 69 kDa
      Observed band size: 69, 70 kDa
    • Immunohistochemistry of paraffin-embedded human colon cancer using CSB-PA887999EA01HU at dilution of 1:100
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) ACAD9 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
  • 別名:
    ACAD9Complex I assembly factor ACAD9 antibody; mitochondrial antibody; Acyl-CoA dehydrogenase family member 9 antibody; ACAD-9 antibody; EC 1.3.8.- antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial protein (1-270AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,ACAD9 Antibody (CSB-PA887999EA01HU),的標記方式是Non-conjugated。對于ACAD9 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-PA887999EB01HU ACAD9 Antibody, HRP conjugated ELISA
    FITC CSB-PA887999EC01HU ACAD9 Antibody, FITC conjugated
    Biotin CSB-PA887999ED01HU ACAD9 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, IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • 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:
    As part of the MCIA complex, primarily participates in the assembly of the mitochondrial complex I and therefore plays a role in oxidative phosphorylation. This moonlighting protein has also a dehydrogenase activity toward a broad range of substrates with greater specificity for long-chain unsaturated acyl-CoAs. However, in vivo, it does not seem to play a primary role in fatty acid oxidation. In addition, the function in complex I assembly is independent of the dehydrogenase activity of the protein.
  • 基因功能參考文獻:
    1. Mutations in the ND6, NDUFV1 or ACAD9 genes are responsible for the mitochondrial complex I deficiency. PMID: 29348607
    2. Study identified new mutations in ACAD9 responsible for a wide spectrum of heart diseases in the presence of elevated serum lactate levels. PMID: 27233227
    3. ACAD9 mutation is the most frequent cause of cardiac hypertrophy and isolated complex I deficiency. PMID: 26669660
    4. Case Report: neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules. PMID: 26826406
    5. In cells where it is strongly expressed, ACAD9 plays a physiological role in fatty acid oxidation. PMID: 25721401
    6. Our results underscore the importance of the ACAD9 protein in complex I assembly and suggest that the enzymatic activity is a rudiment of the duplication event. PMID: 24158852
    7. Our data support a new function for ACAD9 in complex I function, making this gene an important new candidate for patients with complex I deficiency, which could be improved by riboflavin treatment. PMID: 20929961
    8. ACAD9 screening of 120 additional complex I-defective index cases led us to identify two additional unrelated cases and a total of five pathogenic ACAD9 alleles. PMID: 21057504
    9. Data show that two closely related metabolic enzymes, ACAD9 and VLCAD, diverged at the root of the vertebrate lineage to function in two separate mitochondrial metabolic pathways and have clinical implications for the diagnosis of complex I deficiency. PMID: 20816094
    10. Very high activity of CPT2 and VCLAD, involved in the metabolism of long-chain fatty acids. Fatty acid oxidation may play role in energy generation in placenta, and deficiency in may result in placental dysfunction and gestational complications. PMID: 12971426
    11. ACAD9 may play a role in the turnover of lipid membrane unsaturated fatty acids that are essential for membrane integrity and structure PMID: 16020546
    12. acyl-CoA dehydrogenase 9 (ACAD 9)was identified as the long-chain ACAD in human embryonic and fetal brain and central nervous tissue, using in situ hybridization as well as enzymatic studies PMID: 16750164
    13. We now report three cases of ACAD9 deficiency. PMID: 17564966
    14. Accumulation of 3-hydroxylated intermediates of long-chain fatty acids may contribute to the pathogenesis of retinopathy in MTP deficiencies. PMID: 18385088
    15. Validated occurrence of an unusual TG 3' splice site in intron 10. PMID: 17672918

    顯示更多

    收起更多

  • 相關疾病:
    Acyl-CoA dehydrogenase family, member 9, deficiency (ACAD9 deficiency)
  • 亞細胞定位:
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族:
    Acyl-CoA dehydrogenase family
  • 組織特異性:
    Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver. In the cerebellum uniquely expressed in the granular layer (at protein level).
  • 數據庫鏈接:

    HGNC: 21497

    OMIM: 611103

    KEGG: hsa:28976

    STRING: 9606.ENSP00000312618

    UniGene: Hs.567482



主站蜘蛛池模板: 国产精品毛片更新无码| 综合自拍亚洲综合图区高清| 日韩精品无码中文字幕一区二区| 中文字幕丰满乱子伦无码专区| 中国大陆精品视频xxxx | 波多老师无码av中字专区| 亚洲五月天综合| 国产产在线精品亚洲aavv| 麻豆自媒体 一区 二区| 久久久久久国产精品免费免费男同 | 亚洲国产精品特色大片观看完整版 | 精品乱子伦一区二区三区| 亚洲精品伊人久久久大香| 丰满少妇被粗大猛烈进人高清| 中文字幕一二三区波多野结衣| 成人精品一区二区三区中文字幕| 成人精品一区二区三区电影| 色悠久久久久久久综合网| 亚洲aⅴ无码专区在线观看q| 性欧美俄罗斯乱妇| 国产高清不卡免费视频| 久久精品免视看国产成人| 97久久超碰亚洲视觉盛宴| 影音先锋每日av色资源站| 四虎永久在线精品8848a| 国产免费一卡二卡三卡四卡| 欧美私人情侣网站| 久久99精品久久久久久动态图| 午夜精品久久久久久| 国产色无码精品视频免费 | 无遮挡18禁啪啪免费观看| 久久国产精品免费一区| 国产99久9在线视频 | 传媒| 成人看片黄a免费看在线| 国产精品多人p群无码| 又粗又大内射免费视频小说| 大屁股熟女一区二区三区| 极品少妇一区二区三区四区 | 一二三四视频社区在线播放中国| 精品无人区卡一卡二卡三乱码 | 欧美婷婷六月丁香综合色 |