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HADHA Antibody

  • 货号：
    CSB-PA010118GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P40939
  • 基因名：
    HADHA
  • 别名：
    3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody; 3 oxoacyl CoA thiolase antibody; 78 kDa gastrin binding protein antibody; 78 kDa gastrin-binding protein antibody; ECHA antibody; ECHA_HUMAN antibody; GBP antibody; HADH antibody; HADHA antibody; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody; LCEH antibody; LCHAD antibody; Long chain 3-hydroxyacyl-CoA dehydrogenase antibody; Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit antibody; Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit antibody; Mitochondrial trifunctional enzyme alpha subunit antibody; Mitochondrial trifunctional protein alpha subunit antibody; MTPA antibody; Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody; TP ALPHA antibody; TP-alpha antibody; Trifunctional enzyme subunit alpha mitochondrial precursor antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human HADHA
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Mitochondrial trifunctional protein deficiency (MTPD); Long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency); Maternal acute fatty liver of pregnancy (AFLP)
  • 亚细胞定位：
    Mitochondrion. Mitochondrion inner membrane.
  • 蛋白家族：
    Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family
  • 数据库链接：

    HGNC: 4801

    OMIM: 600890

    KEGG: hsa:3030

    STRING: 9606.ENSP00000370023

    UniGene: Hs.516032