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  4. Recombinant Human Trifunctional enzyme subunit alpha, mitochondrial (HADHA)

Recombinant Human Trifunctional enzyme subunit alpha, mitochondrial (HADHA)

  • 中文名稱：
    人HADHA重組蛋白
  • 貨號：
    CSB-YP340324HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人HADHA重組蛋白
  • 貨號：
    CSB-EP340324HU-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：
    人HADHA重組蛋白
  • 貨號：
    CSB-BP340324HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人HADHA重組蛋白
  • 貨號：
    CSB-MP340324HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    HADHA
  • Uniprot No.：
  • 別名：
    3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit; 3 oxoacyl CoA thiolase; 78 kDa gastrin binding protein; 78 kDa gastrin-binding protein; ECHA; ECHA_HUMAN; GBP; HADH; HADHA; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit; LCEH; LCHAD; Long chain 3-hydroxyacyl-CoA dehydrogenase; Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit; Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit; Mitochondrial trifunctional enzyme alpha subunit; Mitochondrial trifunctional protein alpha subunit; MTPA; Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit; TP ALPHA; TP-alpha; Trifunctional enzyme subunit alpha mitochondrial precursor
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達區域：
    37-763
  • 氨基酸序列
    TRTH INYGVKGDVA VVRINSPNSK VNTLSKELHS EFSEVMNEIW ASDQIRSAVL ISSKPGCFIA GADINMLAAC KTLQEVTQLS QEAQRIVEKL EKSTKPIVAA INGSCLGGGL EVAISCQYRI ATKDRKTVLG TPEVLLGALP GAGGTQRLPK MVGVPAALDM MLTGRSIRAD RAKKMGLVDQ LVEPLGPGLK PPEERTIEYL EEVAITFAKG LADKKISPKR DKGLVEKLTA YAMTIPFVRQ QVYKKVEEKV RKQTKGLYPA PLKIIDVVKT GIEQGSDAGY LCESQKFGEL VMTKESKALM GLYHGQVLCK KNKFGAPQKD VKHLAILGAG LMGAGIAQVS VDKGLKTILK DATLTALDRG QQQVFKGLND KVKKKALTSF ERDSIFSNLT GQLDYQGFEK ADMVIEAVFE DLSLKHRVLK EVEAVIPDHC IFASNTSALP ISEIAAVSKR PEKVIGMHYF SPVDKMQLLE IITTEKTSKD TSASAVAVGL KQGKVIIVVK DGPGFYTTRC LAPMMSEVIR ILQEGVDPKK LDSLTTSFGF PVGAATLVDE VGVDVAKHVA EDLGKVFGER FGGGNPELLT QMVSKGFLGR KSGKGFYIYQ EGVKRKDLNS DMDSILASLK LPPKSEVSSD EDIQFRLVTR FVNEAVMCLQ EGILATPAEG DIGAVFGLGF PPCLGGPFRF VDLYGAQKIV DRLKKYEAAY GKQFTPCQLL ADHANSPNKK FYQ
  • 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：
    Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity. Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity. It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP. Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity.
  • 基因功能參考文獻：
    1. study indicate described high frequency of c.1528G>C variant of HADHA gene in Kashubian population, suggesting the founder effect. For the first time we have found high frequency of rs71441018 in the South Poland Silesian population PMID: 29095929
    2. Low HADHA expression is associated with clear cell renal cell carcinoma. PMID: 26715271
    3. Exposure to bezafibrate (400 muM for 48 h) increased the abundance of HADHA and HADHB mRNAs. PMID: 26109258
    4. findings suggest an auxiliary role for HADHA in miRNA biogenesis and help in better understanding of molecular mechanisms underlying RNAi pathway PMID: 26367179
    5. nonstructural protein 5 (NS5) interacted with hydroxyacyl-CoA dehydrogenase alpha and beta subunits, two components of the mitochondrial trifunctional protein (MTP) involved in LCFA beta-oxidation PMID: 25816318
    6. revealed a novel G/A allelic change in the intronic region [chromosomal position 26417674; 20 bp adjacent to an already published SNP (rs151243950)] for one among the three acute fatty liver of pregnancy patients PMID: 24105666
    7. Crystal structures of human mitochondrial 3-ketoacyl-CoA thiolase (hT1) in the apo form and in complex with CoA have been determined at 2.0 A resolution. The structures confirm the tetrameric quaternary structure of this degradative thiolase. PMID: 25478839
    8. Mitochondrial Trifunctional-Protein depletion during hepatitis c virus infection rendered cells less responsive to alpha interferon treatment by impairing IFN-stimulated gene expression. PMID: 25673715
    9. Report probable Kashubian origin of the prevalent HADHA c.1528G>C mutation responsible for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency in Poland. PMID: 20814823
    10. Free fatty acids might affect the expression of mitochondrial beta-oxidation enzyme of LCHAD in trophoblast cells. PMID: 23253803
    11. mitochondrial alphaTFP exhibits both in vitro and in vivo MLCL AT activity linking an enzyme of mitochondrial beta-oxidation to Cardiolipin remodeling. PMID: 23152787
    12. these findings lend support to the hypothesis that mutations in the HADHA gene may be directly associated with and potentially causative of Maternal floor infarction/massive perivillous fibrin deposition PMID: 22746996
    13. TP-alpha, collagen alpha-1(VI) chain and S100A9 are potential biomarkers of esophageal squamous cell carcinoma, and may play an important role in tumorigenesis and development of ESCC. PMID: 22583932
    14. Both children were homozygous for the common mutation c.1528G>C and the parents were heterozygous for this same mutation. PMID: 22325456
    15. High HADHA protein expression is associated with response to platinum-based chemotherapy for lung cancer. PMID: 22471497
    16. Results emphasize the value of cDNA analysis in the characterization of HADHA and HADHB mutations and further strengthen the model of haploinsufficiency as a major pathomechanism in MTP defects. PMID: 21549624
    17. Recombinant mitochondrial trifunctional protein displayed 2-enoyl-CoA hydratase, l-3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase activities. PMID: 20825197
    18. The G1528C (E474Q) mutation in the alpha-subunit of the mitochondrial trifunctional protein was not found in 10 women with acute fatty liver of pregnancy. PMID: 11978893
    19. Plasma concentration varied more than erythrocyte levels. Postprandial period and fasting. PMID: 12971430
    20. Both alpha- and beta-subunit mutations result in TFP complex instability, demonstrating that the mechanism of disease is the same in alpha- or beta-mutation-derived disease and explaining the biochemical and clinical similarities. PMID: 14630990
    21. The retinal pigment epithelium rather than the choriocapillaris could be the critical affected cell layer in LCHAD retinopathy PMID: 15347768
    22. DNA analysis of patients 1 and 2 revealed homozygosity for a c.1689+2T>G mutation of the HADHA gene, resulting in the skipping of exon 16 with an in-frame 69-bp deletion. PMID: 17143551
    23. Neither maternal nor fetal heterozygosity for the E474Q mutation is a relevant factor of HELLP syndrome. PMID: 17313315
    24. There might be diverse etiological factors in China contributing to AFLP other than the frequently reported mutation in the LCHAD. PMID: 18031367
    25. The second of 220 SIDs cases was a compound heterozygous for the prevalent MTP G1528C mutation and a novel 1 bp deletion in exon 18 of the MTPalpha-subunit gene. PMID: 18045290
    26. Severe cardiac mitochondrial proliferation and TFP deficiency was observed in intrauterine cardiomyopathy. PMID: 18485779
    27. identify MLCL AT-1 as a human mitochondrial monolysocardiolipin acyltransferase involved in the remodeling of cardiolipin PMID: 19737925

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  • 相關疾病：
    Mitochondrial trifunctional protein deficiency (MTPD); Long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency); Maternal acute fatty liver of pregnancy (AFLP)
  • 亞細胞定位：
    Mitochondrion. Mitochondrion inner membrane.
  • 蛋白家族：
    Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family
  • 數據庫鏈接：

    HGNC: 4801

    OMIM: 600890

    KEGG: hsa:3030

    STRING: 9606.ENSP00000370023

    UniGene: Hs.516032



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