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： 国产suv精品一区二区69| 亚洲精品国偷自产在线99人热 | 国产成人亚洲综合网色欲网| 人人澡人人妻人人爽人人蜜桃麻豆| 国产尤物精品自在拍视频首页| 亚洲综合色视频在线观看| 中文字幕人妻熟女在线| 亚洲精品久久区二区三区蜜桃臀| 无码人妻精品中文字幕不卡| 国产精品 中文字幕 亚洲 欧美| 无码人妻精品一二三区免费| 国产人妖xxxx做受视频| 成av人电影在线观看| 中文字幕在线亚洲日韩6页| 三个男人躁我一个爽视频免费| 中文字幕人成人乱码亚洲电影| 艳妇乳肉豪妇荡乳| 亚洲中文字幕无码人在线| 国产片免费福利片永久| 亚洲人ⅴsaⅴ国产精品| 日日干夜夜干| 国产欧美另类久久久精品图片| 亚洲国产成人综合在线不卡| 久久精品久久精品久久39| 久久精品丝袜高跟鞋| 正在播放的国产a一片| 亚洲色无码专区一区| 久久久久av无码免费网| 日韩成人无码一区二区三区| 中文字幕av中文字无码亚| 亚洲欧美在线观看品| 精品国产一区二区三区久久| 日韩免费无码一区二区视频| 亚洲 日韩 欧美 成人 在线| 2019最新中文字幕在线观看| 成 人 免费观看网站| 国产v亚洲v天堂无码久久久| 日韩人妻无码免费视频一二区| 欧美国产日韩a在线视频下载| 影音先锋人妻啪啪av资源网站| 中文字字幕在线中文无码|