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

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. ACAD9 Antibody

ACAD9 Antibody

  • 中文名稱：
    ACAD9兔多克隆抗體
  • 貨號：
    CSB-PA055934
  • 規格：
    ￥1100
  • 圖片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using CSB-PA055934(ACAD9 Antibody) at dilution 1/15, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA055934(ACAD9 Antibody) at dilution 1/15, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

產品詳情

  • 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：
    Fusion protein of Human ACAD9
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen affinity purification
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA,IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    IHC 1:15-1:50
  • 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： 亚洲卡1卡2卡新区网站| 男女做爰猛烈叫床视频动态图| 欧美成人午夜免费影院手机在线看| 中文乱码免费一区二区三区| 亚洲爆乳大丰满无码专区| 国产精品久久久久久久久岛| 国产白丝无码免费视频| 久久人人爽人人爽爽久久小说| 日韩99在线 | 中文| 成人久久久久久久久久久| 久久久久人妻精品一区二区三区| 亚洲福利国产网曝| 国产免费人成视频在线播放播| 亚洲色中文字幕在线播放| 欧美综合在线激情专区| 自拍偷自拍亚洲精品被多人伦好爽| 丰满少妇弄高潮了www| 欲色影视天天一区二区三区色香欲| 在线人成免费视频69国产| 无码丰满熟妇bbbbxxx| 野外少妇愉情中文字幕| 男女啪祼交视频| 亚洲 欧美 另类 制服 日韩| 亚洲精品无码久久久久不卡| 国产精品.xx视频.xxtv| 人妻另类 专区 欧美 制服| 午夜a理论片在线播放| 成 人影片 免费观看在线| 久久国产色av免费观看| 亚洲日本一区二区一本一道| 日韩丝袜欧美人妻制服| 日本高清在线www3344| 国产免费一区二区三区在线观看| 国产色视频一区二区三区 | 久久久久久无码午夜精品直播| 自拍性旺盛老熟女| 国产色产综合色产在线视频| av无码中文字幕不卡一区二区三区 | 国产在线视频精品视频| 午夜免费啪视频在线观看区| 日韩欧美亚洲国产精品字幕久久久|