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-PA948378
  • 規格：
    ￥1100
  • 圖片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using CSB-PA948378(ACAD9 Antibody) at dilution 1/10, 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-PA948378(ACAD9 Antibody) at dilution 1/10, 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 1:2000-1:5000
    IHC 1:25-1:100
  • 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： 少妇的肉体aa片免费| 开心色怡人综合网站| 99精品视频在线观看免费蜜桃| 好吊妞国产欧美日韩免费观看| 亚洲第一综合天堂另类专| 国产精品最新免费视频| 小草国产精品情侣| 好吊色欧美一区二区三区四区 | 狼群社区www中文视频| 无码专区丰满人妻斩六十路| 中国极品少妇videossexhd| 99精品久久99久久久久| 亚洲韩国日本高清一区| 嘿咻嘿咻男女免费专区| 伊人久久大香线蕉av色婷婷色| 高潮毛片无遮挡高清视频播放| 亚洲一区天堂九一| 亚洲伊人成综合网2222| 国产亚洲精品久久久久久久久动漫| 一出一进一爽一粗一大视频免费的| 亚洲在av极品无码| 极品老师腿张开粉嫩小泬| 美女裸体色黄污视频网站| 欧洲熟妇色xxxxx视频| 高潮潮喷奶水飞溅视频无码| а天堂中文在线官网| 欧美色就是色| 无码av中文一区二区三区| 成视频年人黄网站免费视频 | 欧美丰满一区二区免费视频| 欧美人禽杂交狂配免费看| 伊人久久精品av一区二区| 国产午夜精品一区理论片飘花 | 亚洲综合久久精品无码色欲| 中国国产免费毛卡片| 国产免费福利在线视频| 精品国产av一区二区三区| 久久亚洲国产成人影院| 亚洲亚洲人成无码网www| 一本加勒比hezyo无码人妻| 人妻少妇精品无码专区|