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 Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADVL)

Recombinant Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADVL)

  • 中文名稱：
    Recombinant Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADVL)
  • 貨號：
    CSB-YP001129HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    Recombinant Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADVL)
  • 貨號：
    CSB-EP001129HU
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    Recombinant Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADVL)
  • 貨號：
    CSB-EP001129HU-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 Very long-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADVL)
  • 貨號：
    CSB-BP001129HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    Recombinant Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial(ACADVL)
  • 貨號：
    CSB-MP001129HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    ACADVL
  • Uniprot No.：
  • 別名：
    ACAD 6; ACAD6; ACADV_HUMAN; Acadvl; Acyl CoA dehydrogenase very long chain; Acyl Coenzyme A dehydrogenase very long chain; LCACD; mitochondrial; Very long chain specific acyl CoA dehydrogenase; Very long chain specific acyl CoA dehydrogenase mitochondrial; Very long-chain specific acyl-CoA dehydrogenase; VLCAD
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full length protein
  • 表達區域：
    41-655
  • 氨基酸序列
    AGGAAQLALD KSDSHPSDAL TRKKPAKAES KSFAVGMFKG QLTTDQVFPY PSVLNEEQTQ FLKELVEPVS RFFEEVNDPA KNDALEMVEE TTWQGLKELG AFGLQVPSEL GGVGLCNTQY ARLVEIVGMH DLGVGITLGA HQSIGFKGIL LFGTKAQKEK YLPKLASGET VAAFCLTEPS SGSDAASIRT SAVPSPCGKY YTLNGSKLWI SNGGLADIFT VFAKTPVTDP ATGAVKEKIT AFVVERGFGG ITHGPPEKKM GIKASNTAEV FFDGVRVPSE NVLGEVGSGF KVAMHILNNG RFGMAAALAG TMRGIIAKAV DHATNRTQFG EKIHNFGLIQ EKLARMVMLQ YVTESMAYMV SANMDQGATD FQIEAAISKI FGSEAAWKVT DECIQIMGGM GFMKEPGVER VLRDLRIFRI FEGTNDILRL FVALQGCMDK GKELSGLGSA LKNPFGNAGL LLGEAGKQLR RRAGLGSGLS LSGLVHPELS RSGELAVRAL EQFATVVEAK LIKHKKGIVN EQFLLQRLAD GAIDLYAMVV VLSRASRSLS EGHPTAQHEK MLCDTWCIEA AARIREGMAA LQSDPWQQEL YRNFKSISKA LVERGGVVTS NPLGF
  • 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：
    Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, very long-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains.
  • 基因功能參考文獻：
    1. There are currently no biochemical markers for prediction of disease severity and for the need for treatment in VLCAD deficiency. Mutation analysis may offer predictive value but this may not be robust enough for a large proportion of those mutations that have not been previously reported in clinically affected patients. PMID: 27246109
    2. We retrospectively analyzed early outcomes for individuals who were diagnosed with VLCAD deficiency by NBS and describe initial presentations, diagnosis, clinical outcomes and treatment in a cohort of 52 individuals ages 1-18year. PMID: 27209629
    3. following variants should be considered likely pathogenic c.1273G > A (p.A425T), c.1001T > G (p.M334R), c.538G > A (p.A180T), c.640T > G (p.F214V), c.1076C > T (p.A359V), c.1019G > T (p.G340V), c.889_891delGAG (p.E297del), and c.1103A > C (p.Q368P); patients homozygous for the most common pathogenic variant, c.848T > C (p.V283A) can be expected to have a more benign clinical course PMID: 26385305
    4. 11 mutations in ACADVL gene in 7 patients, 7 reported (p.S22X, p.W427X, p.A213T, p.G222R, p.R450H, c.296-297delCA, c.1605+1G>T), 4 novel (p.S72F, p.Q100X, p.M437T, p.D466Y). p.R450H and p.D466Y (14.28%, 2/14 alleles) mutations identified in 2 alleles. PMID: 24801231
    5. Case Report: missense mutation within the ACADVL gene responsible for very-long-chain acyl-CoA dehydrogenase deficiency and sudden infant death. PMID: 20107901
    6. These results emphasize the importance of functional investigation of abnormal NBS or clinical testing suggestive but not diagnostic of very-long-chain acyl-CoA dehydrogenase . PMID: 23480858
    7. These findings support the importance of considering that mutations may be present in the ACADVL gene when a significant partial deficiency is found in CPTII activity, but no mutations in the CPT2 gene can be identified. PMID: 23169530
    8. Identification of 2 VLCAD mutations leads to precautions in the management of the children with VLCAD deficiency. PMID: 21932095
    9. The expressions of LCHAD gene and protein are remarkably reduced in early onset severe preeclampsia and HELLP syndrome. PMID: 22093928
    10. Analyzed potential rhabdomyolysis-susceptibility genes (RYR 1, CPT II, VLCAD and CYP 2D6) from autopsy samples of methamphetamine abusers; no obvious relationship between the genetic mutations observed in this study and rhabdomyolysis was seen. PMID: 20952238
    11. Down regulation of ACADVL is associated with cervical squamous cell carcinoma. PMID: 20099975
    12. Missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase is associated with inborn errors of lipid metabolism. PMID: 20060901
    13. This study confirms that VLCAD deficiency, although being less frequent than CPT II deficiency, should be systematically considered in the differential diagnosis of exercise-induced rhabdomyolysis. PMID: 19327992
    14. Results suggest a novel regulatory mechanism for homeostatic VLCAD activity, whose dysregulation might be involved in the production of oxidative stress and in the pathogenesis of idiopathic pulmonary fibrosis. PMID: 19889959
    15. A new a unique mutation (IVS13+25G>A) is reported in a compound heterozygote carrying the 1748 C>T mutation in exon 18. PMID: 16464760
    16. the bacterial expression system developed here will significantly advance our understanding of both the clinical aspects of VLCAD deficiency and the basic biochemistry of the enzyme PMID: 17374501
    17. In asymptomatic mild VLCADdeficiency, a fat-reduced diet may not be necessary, whereas in later infancy and adolescence, strenuous physical exercise may require additional energy from medium-chain fat. PMID: 17457695
    18. Report the course of disease in a pair of monozygotic twin sisters. PMID: 17514507
    19. Bezafibrate, a widely prescribed hypolipidemic drug, cn be used for the correction of VLCAD deficiency and exemplifies the integration of molecular information in a therapeutic strategy PMID: 17999356
    20. Loss of heterozygosity on 17p13 and down-regulation of ACADVL can be used to discriminate adrenal cortex neoplasms from adrenocortical adenoma. PMID: 18156936

    顯示更多

    收起更多

  • 相關疾?。?/div>
    Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD)
  • 亞細胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein.; [Isoform 2]: Mitochondrion inner membrane; Peripheral membrane protein.
  • 蛋白家族：
    Acyl-CoA dehydrogenase family
  • 數據庫鏈接：

    HGNC: 92

    OMIM: 201475

    KEGG: hsa:37

    STRING: 9606.ENSP00000349297

    UniGene: Hs.437178



主站蜘蛛池模板： 中文字字幕在线中文乱码| 男女猛烈xx00免费视频试看| 久久国产色av免费看| 日本高清熟妇老熟妇| 韩国三级hd中文字幕叫床| 国产精品va在线观看丝瓜影院| 国产精品人妻一区二区高| 久久综合亚洲色一区二区三区| yw尤物av无码国产在线观看| 精品国产无套在线观看| 国产最新av在线播放不卡| 最近中文av字幕在线中文| 久久五月精品中文字幕| 国产亚洲精品a在线| 亚洲一卡一卡二新区迎热烈讨论| 欧美午夜成人片在线观看| 午夜亚洲www湿好爽| 亚洲人成影院在线无码按摩店| 男女下面一进一出好爽视频| 成人午夜亚洲精品无码区| 18禁肉肉无遮挡无码网站| 亚洲色无码专区在线观看精品| 国产精品一区二区手机在线观看 | 亚洲一区二区三区日本久久九| 亚洲国产中文字幕在线视频综合| 久久婷婷五月综合色欧美| 蜜臀久久99精品久久久久久做爰| 天堂资源在线官网| 午夜大片免费男女爽爽影院| 国产免费无码一区二区视频| 亚洲中文字幕伊人久久无码| 2020无码天天喷水天天爽| 东北少妇不带套对白| 日韩精品无码中文字幕一区二区| 无码免费中文字幕视频| 97久久综合亚洲色hezyo| 88国产精品欧美一区二区三区| 久久永久免费专区人妻精品| 国产无遮挡又黄又爽高潮| 中文在线√天堂| 久久综合精品国产一区二区三区无码|