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. 首頁
  2. 蛋白
  3. 重組蛋白
  4. Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial (ACAD9), partial

Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial (ACAD9), partial

  • 中文名稱：
    Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial(ACAD9) ,partial
  • 貨號：
    CSB-YP887999HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial(ACAD9) ,partial
  • 貨號：
    CSB-EP887999HU
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial(ACAD9) ,partial
  • 貨號：
    CSB-EP887999HU-B
  • 規格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial(ACAD9) ,partial
  • 貨號：
    CSB-BP887999HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial(ACAD9) ,partial
  • 貨號：
    CSB-MP887999HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 別名：
    ACAD9Complex I assembly factor ACAD9; mitochondrial; Acyl-CoA dehydrogenase family member 9; ACAD-9; EC 1.3.8.-
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Partial
  • 表達區域：
    -
  • 蛋白標簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

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： 性xxxx搡xxxxx搡欧美| 亚洲 制服丝袜 中文字幕 在线| 日本边添边摸边做边爱边| 亚洲人成网站在小说| 欧美大荫蒂毛茸茸视频| 狠狠88综合久久久久综合网| 国产成人小视频| 亚洲精品无码av黄瓜影视| 亚色九九九全国免费视频| 人人狠狠综合久久亚洲爱咲| 国产成人免费一区二区三区| 欧美性猛交xxxx乱大交极品| 99在线精品一区二区三区| 亚洲欧美国产国产一区二区三区| 日本高清一区二区三| 欧美乱妇高清无乱码在线观看| 囯产精品久久久久久久久久妞妞| 国产女精品视频网站免费| 久久亚洲精品无码av宋| av大尺度一区二区三区| 蜜臀av无码人妻精品| 久久婷婷五月综合色国产香蕉| 精品国内综合一区二区| 亚洲人成黄网站69影院| 欧美午夜一区二区福利视频| 一区二区三区国产亚洲网站| 无翼乌工口全彩肉肉无遮挡18 | 成人看片黄a免费看在线| 日韩美女自卫慰黄网站| 久久青青草免费线频观| 爱做久久久久久| 在线观看国产成人av片| 少妇无码av无码一区| 狠狠色丁香久久综合婷婷| julia无码中文字幕一区| 亚洲女教师丝祙在线播放| 一本无码中文字幕在线观| 在线观看特色大片免费视频 | 亚洲精品国产suv一区88| 国产女人精品视频国产灰线| 清纯唯美经典一区二区|