Gene Symbol: HADHA
Description: hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
Alias: ECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA, trifunctional enzyme subunit alpha, mitochondrial, 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit, 3-oxoacyl-CoA thiolase, 78 kDa gastrin-binding protein, gastrin-binding protein, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit, hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit, long-chain 2-enoyl-CoA hydratase, 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 (CoA) dehydrogenase, alpha subunit, mitochondrial trifunctional enzyme, alpha subunit, mitochondrial trifunctional protein, alpha subunit
Species: human
Products:     HADHA

Top Publications

  1. Taylor W, Hatch G. Identification of the human mitochondrial linoleoyl-coenzyme A monolysocardiolipin acyltransferase (MLCL AT-1). J Biol Chem. 2009;284:30360-71 pubmed publisher
    ..The results identify MLCL AT-1 as a human mitochondrial monolysocardiolipin acyltransferase involved in the remodeling of cardiolipin. ..
  2. Carpenter K, Pollitt R, Middleton B. Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria. Biochem Biophys Res Commun. 1992;183:443-8 pubmed
    ..coli, but unique in its membrane location and substrate specificity. We propose that its existence explains the repeated failure to detect any intermediates of mitochondrial beta-oxidation. ..
  3. Brackett J, Sims H, Rinaldo P, Shapiro S, Powell C, Bennett M, et al. Two alpha subunit donor splice site mutations cause human trifunctional protein deficiency. J Clin Invest. 1995;95:2076-82 pubmed
    ..This is the initial molecular characterization of trifunctional protein deficiency. ..
  4. Kamijo T, Wanders R, Saudubray J, Aoyama T, Komiyama A, Hashimoto T. Mitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients. J Clin Invest. 1994;93:1740-7 pubmed
    ..Taken together, the results obtained show that in both patients, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is caused by an abnormality in the trifunctional protein, even though there is a heterogeneity in both patients. ..
  5. Liao C, Lin Y, Kuo C. Effect of high-fat diet on hepatic proteomics of hamsters. J Agric Food Chem. 2015;63:1869-81 pubmed publisher
    ..05). The α-subunit of hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (HADHA), which catalyzes the second and third reactions of β-oxidation, was down-regulated by the high-fat diet (p <..
  6. Clotet S, Soler M, Riera M, Pascual J, Fang F, Zhou J, et al. Stable Isotope Labeling with Amino Acids (SILAC)-Based Proteomics of Primary Human Kidney Cells Reveals a Novel Link between Male Sex Hormones and Impaired Energy Metabolism in Diabetic Kidney Disease. Mol Cell Proteomics. 2017;16:368-385 pubmed publisher
    ..glucosamine-6-phosphate-N-acetyltransferase 1 (GNPNAT1), and mitochondrial trifunctional protein subunit alpha (HADHA), were verified in vitro...
  7. Frampton G, Jones J, Rose M, Payne L. Placental growth factor (alone or in combination with soluble fms-like tyrosine kinase 1) as an aid to the assessment of women with suspected pre-eclampsia: systematic review and economic analysis. Health Technol Assess. 2016;20:1-160 pubmed
    ..The model evaluates costs (2014, GBP) from a NHS and Personal Social Services perspective...
  8. Martin G, Atshaves B, McIntosh A, Mackie J, Kier A, Schroeder F. Liver fatty acid-binding protein gene-ablated female mice exhibit increased age-dependent obesity. J Nutr. 2008;138:1859-65 pubmed
    ..The data support a working model in which obesity development in these mice results from shifts toward reduced energy expenditure and/or more efficient energy uptake in the gut. ..
  9. Spiekerkoetter U, Wood P. Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models. J Inherit Metab Dis. 2010;33:539-46 pubmed publisher
    ..low platelets (HELLP) syndrome, as observed in pregnancies carrying a long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD)-deficient fetus...

More Information

Publications140 found, 100 shown here

  1. Wang Q, Liu M, Xu L, Wu Y, Huang Y. Transcriptome analysis reveals the molecular mechanism of hepatic fat metabolism disorder caused by Muscovy duck reovirus infection. Avian Pathol. 2018;47:127-139 pubmed publisher
    ..FDR: false discovery rate; GCDH: Pseudopodoces humilis glutaryl-CoA dehydrogenase; GO: Gene Ontology; HADHA: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; ..
  2. Asghari A, Marashi S, Ansari Pour N. A sperm-specific proteome-scale metabolic network model identifies non-glycolytic genes for energy deficiency in asthenozoospermia. Syst Biol Reprod Med. 2017;63:100-112 pubmed publisher
    ..FH: fumarate hydratase; IDH: isocitrate dehydrogenase; SUCLG1: succinate-CoA ligase; SD: succinate dehydrogenase; HADHA: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase, subunit A; HADHB: hydroxyacyl-CoA ..
  3. Guo X, Zhang H, Zheng X, Zhou Q, Yang Y, Chen X, et al. Structural and functional characterization of a novel gene, Hc-daf-22, from the strongylid nematode Haemonchus contortus. Parasit Vectors. 2016;9:422 pubmed publisher
    ..elegans as a model organism. Our results indicate that Hc-daf-22 shared similar characteristics and function with Ce-daf-22 and may play an important role in peroxisomal ?-oxidation and the development in H. contortus. ..
  4. Spiekerkoetter U. Mitochondrial fatty acid oxidation disorders: clinical presentation of long-chain fatty acid oxidation defects before and after newborn screening. J Inherit Metab Dis. 2010;33:527-32 pubmed publisher
    ..trifunctional protein (TFP) complex, including long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase (LCHAD) deficiency, neuropathy and retinopathy develop that are progressive and irreversible despite current treatment ..
  5. De Biase I, Viau K, Liu A, Yuzyuk T, Botto L, Pasquali M, et al. Diagnosis, Treatment, and Clinical Outcome of Patients with Mitochondrial Trifunctional Protein/Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency. JIMD Rep. 2017;31:63-71 pubmed publisher
    Deficiency of the mitochondrial trifunctional protein (TFP) and long-chain 3-Hydroxy Acyl-CoA dehydrogenase (LCHAD) impairs long-chain fatty acid oxidation and presents with hypoglycemia, cardiac, liver, eye, and muscle involvement...
  6. Fabozzi M, Brachet Contul R, Millo P, Allieta R. Intestinal infarction by internal hernia in Petersen's space after laparoscopic gastric bypass. World J Gastroenterol. 2014;20:16349-54 pubmed publisher
    Intestinal occlusion by internal hernia is not a rare complication (0.2%-5%) after Laparoscopic Roux-en-Y-GBP (LGBP) with higher morbidity and mortality related to mesenteric vessels involvement...
  7. Deng Q, Li X, Fu S, Yin L, Zhang Y, Wang T, et al. SREBP-1c gene silencing can decrease lipid deposits in bovine hepatocytes cultured in vitro. Cell Physiol Biochem. 2014;33:1568-78 pubmed publisher
    ..carnitine palmitoyltransferase І (CPT-І), carnitine palmitoyltransferase II (CPT- II), and β-hydroxyacyl-CoA-DH (HADH) were significantly elevated...
  8. Frankl Vilches C, Kuhl H, Werber M, Klages S, Kerick M, Bakker A, et al. Using the canary genome to decipher the evolution of hormone-sensitive gene regulation in seasonal singing birds. Genome Biol. 2015;16:19 pubmed publisher
    ..We present the high quality assembly and annotation of a female 1.2-Gbp canary genome...
  9. Wende A, Kim J, Holland W, Wayment B, O Neill B, Tuinei J, et al. Glucose transporter 4-deficient hearts develop maladaptive hypertrophy in response to physiological or pathological stresses. Am J Physiol Heart Circ Physiol. 2017;313:H1098-H1108 pubmed publisher
    ..The requirement for GLUT4 may extend beyond glucose uptake to include defects in mitochondrial metabolism and survival signaling pathways that develop in its absence. Therefore, GLUT4 is critical for responses to hemodynamic stresses. ..
  10. Vennix S, Pelzers L, Bouvy N, Beets G, Pierie J, Wiggers T, et al. Laparoscopic versus open total mesorectal excision for rectal cancer. Cochrane Database Syst Rev. 2014;:CD005200 pubmed publisher
    ..The costs were higher for LTME with differences up to GBP 2000 for direct costs only...
  11. Zhao X, Zhang F, Wang Y. Proteomic analysis reveals Xuesaitong injection attenuates myocardial ischemia/reperfusion injury by elevating pyruvate dehydrogenase-mediated aerobic metabolism. Mol Biosyst. 2017;13:1504-1511 pubmed publisher
    ..expression of 7 proteins, including pyruvate dehydrogenase E1 alpha (PDHA1), hydroxyacyl-coenzyme A dehydrogenase (HADHA), peroxiredoxin 3 (PRX3), gamma-enolase, acetyl-coenzyme A acyltransferase 2 (ACAA2), etc...
  12. Chen X, Huang Z, Ran W, Liao G, Zha L, Wang Z. Type 2 diabetes mellitus control and atherosclerosis prevention in a non-obese rat model using duodenal-jejunal bypass. Exp Ther Med. 2014;8:856-862 pubmed
    ..Roux-en-Y gastric bypass (GBP) is able to induce the remission of T2DM...
  13. Ashmore T, Roberts L, Morash A, Kotwica A, Finnerty J, West J, et al. Nitrate enhances skeletal muscle fatty acid oxidation via a nitric oxide-cGMP-PPAR-mediated mechanism. BMC Biol. 2015;13:110 pubmed publisher
    ..In C2C12 myotubes, nitrate increased expression of the PPARα targets Cpt1b, Acadl, Hadh and Ucp3, and enhanced oxidative phosphorylation rates with palmitoyl-carnitine; however, these changes in gene ..
  14. Lindner M, Hoffmann G, Matern D. Newborn screening for disorders of fatty-acid oxidation: experience and recommendations from an expert meeting. J Inherit Metab Dis. 2010;33:521-6 pubmed publisher
    ..acyl-CoA dehydrogenase deficiency (VLCAD) and long-chain 3-hydroxy acyl-CoA dehydrogenase deficiency (LCHAD) being additional candidates...
  15. Lawrence J, Li Y, Chen S, DeLuca J, Berger J, Umbenhauer D, et al. Differential gene regulation in human versus rodent hepatocytes by peroxisome proliferator-activated receptor (PPAR) alpha. PPAR alpha fails to induce peroxisome proliferation-associated genes in human cells independently of the level of receptor exp. J Biol Chem. 2001;276:31521-7 pubmed
    ..These results suggest that other factors besides PPAR alpha levels determine the species-specific response of human and rat hepatocytes to the induction of PP...
  16. Nemati R, Lu J, Tura A, Smith G, Murphy R. Acute Changes in Non-esterified Fatty Acids in Patients with Type 2 Diabetes Receiving Bariatric Surgery. Obes Surg. 2017;27:649-656 pubmed publisher
    ..and insulin resistance in obese patients with type 2 diabetes (T2D) who underwent laparoscopic gastric bypass (GBP), laparoscopic sleeve gastrectomy (SG) or very low calorie diet (VLCD)...
  17. Li C, Zhu Y, Yu Y, Zhao Q, Wang S, Wang X, et al. Global transcriptome and gene regulation network for secondary metabolite biosynthesis of tea plant (Camellia sinensis). BMC Genomics. 2015;16:560 pubmed publisher
    ..A total of 43.7 Gbp of raw sequencing data were generated, from which 347,827 unigenes were assembled and annotated...
  18. Liu H, Zhang H, Cui R, Guo X, Wang D, Dai J. Activation of peroxisome proliferator-activated receptor α ameliorates perfluorododecanoic acid-induced production of reactive oxygen species in rat liver. Arch Toxicol. 2016;90:1383-97 pubmed publisher
    ..Among them, six significantly changed proteins (CTE1, MTE1, HADHA, ECH1, ALDH2 and CPS1) were found to be regulated by peroxisome proliferator-activated receptor alpha (PPARα)...
  19. Sanchez Ruderisch H, Queirós A, Fliegner D, Eschen C, Kararigas G, Regitz Zagrosek V. Sex-specific regulation of cardiac microRNAs targeting mitochondrial proteins in pressure overload. Biol Sex Differ. 2019;10:8 pubmed publisher
    ..e., Auh, Crat, Decr1, Hadha, and Ndufs4...
  20. Chen X, Li Z, Zhang B, Hu R, Li J, Feng M, et al. Alleviation of Mechanical Allodynia by 14,15-Epoxyeicosatrienoic Acid in a Central Poststroke Pain Model: Possible Role of Allopregnanolone and δ-Subunit-Containing Gamma-Aminobutyric Acid A Receptors. J Pain. 2018;: pubmed publisher
    ..PERSPECTIVE: Agents targeting EETs may serve as potential therapeutic options for stroke, the use of which at the initial period could not only block further nerve damage but also prevent the occurrence of CPSP. ..
  21. Goo Y, Son S, Yechoor V, Paul A. Transcriptional Profiling of Foam Cells Reveals Induction of Guanylate-Binding Proteins Following Western Diet Acceleration of Atherosclerosis in the Absence of Global Changes in Inflammation. J Am Heart Assoc. 2016;5:e002663 pubmed publisher
    ..The interferon-inducible GTPases, guanylate-binding proteins (GBP)3 and GBP6, were induced in the 14-week WD group, and other GBP family members were moderately increased...
  22. Althonaian N, Alsultan A, Morava E, Alfadhel M. Secondary Hemophagocytic Syndrome Associated with COG6 Gene Defect: Report and Review. JIMD Rep. 2018;42:105-111 pubmed publisher
    ..cobalamin C defect, galactosialidosis, Pearson syndrome, and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency...
  23. Yanus G, Akhapkina T, Whitehead A, Bizin I, Iyevleva A, Kuligina E, et al. Exome-based search for recurrent disease-causing alleles in Russian population. Eur J Med Genet. 2019;: pubmed publisher
    ..844G > T (non-classical form of adrenogenital syndrome), EYS c.1155T > A (retinitis pigmentosa), HADHA c.1528G > C (LCHAD deficiency), SCO2 c.418G > A (cytochrome c oxidase deficiency), OTOA c...
  24. Ferreira D, Fiamoncini J, Prist I, Ariga S, de Souza H, de Lima T. Novel role of TLR4 in NAFLD development: Modulation of metabolic enzymes expression. Biochim Biophys Acta. 2015;1851:1353-9 pubmed publisher
    ..enhanced the expression of several enzymes involved in the oxidation of fatty acids, as follows: ACOX, CPT-1, MTPa, MTBb, PBE and 3-ketoacyl-CoA thiolase...
  25. Mohamed Z, Arya V, Hussain K. Hyperinsulinaemic hypoglycaemia:genetic mechanisms, diagnosis and management. J Clin Res Pediatr Endocrinol. 2012;4:169-81 pubmed publisher
    ..Mutations in eight different genes (ABCC8, KCNJ11, GLUD1, CGK, HADH, SLC16A1, HNF4A and UCP2) have been identified to date in patients with congenital forms of hyperinsulinism (CHI)...
  26. Cecatto C, Godoy K, da Silva J, Amaral A, Wajner M. Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle. Toxicol In Vitro. 2016;36:1-9 pubmed publisher
    ..in patients with mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiencies is still unknown...
  27. Lund A, Skovby F, Vestergaard H, Christensen M, Christensen E. Clinical and biochemical monitoring of patients with fatty acid oxidation disorders. J Inherit Metab Dis. 2010;33:495-500 pubmed publisher
    ..ultrasonography in some patients with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase/tri-functional protein (LCHAD/TFP) deficiencies. Biochemical follow-up includes determination of free carnitine and acylcarnitines...
  28. Smith M, Woodhead J, Handy L, Pruess T, Vanegas F, Grussendorf E, et al. Preclinical Comparison of Mechanistically Different Antiseizure, Antinociceptive, and/or Antidepressant Drugs in a Battery of Rodent Models of Nociceptive and Neuropathic Pain. Neurochem Res. 2017;42:1995-2010 pubmed publisher
    ..5 and 15 mg/kg), gabapentin (GBP, 30 and 70 mg/kg), tiagabine (TGB, 5 and 15 mg/kg), and acetominophen (APAP, 250 and 500 mg/kg) reduced ..
  29. Zhao H, Li T, Zhao Y, Tan T, Liu C, Liu Y, et al. Single-cell transcriptomics of human oocytes: environment-driven metabolic competition and compensatory mechanisms during oocyte maturation. Antioxid Redox Signal. 2018;: pubmed publisher
    ..Two of them, ACAT1 and HADHA, were closely related to the regulation of substrate production in the Krebs cycle...
  30. Van Zee J, Schlueter J, Schlueter S, Dixon P, Sierra C, Hill C. Paralog analyses reveal gene duplication events and genes under positive selection in Ixodes scapularis and other ixodid ticks. BMC Genomics. 2016;17:241 pubmed publisher
    ..The haploid genomes of multiple species of ixodid ticks exceed 1 Gbp, prompting questions regarding gene, segmental and whole genome duplication in this phyletic group...
  31. Sevik M, Dogan M. Epidemiological and Molecular Studies on Lumpy Skin Disease Outbreaks in Turkey during 2014-2015. Transbound Emerg Dis. 2017;64:1268-1279 pubmed publisher
    ..The financial cost of disease presence in surveyed cattle farms was estimated to be 72.75 GBP per head...
  32. Matsumi Y, Fujita K, Takashima Y, Yanagida K, Morikawa Y, Matsumoto Nakano M. Contribution of glucan-binding protein A to firm and stable biofilm formation by Streptococcus mutans. Mol Oral Microbiol. 2015;30:217-26 pubmed publisher
    ..The contribution of Gbp expression to biofilm formation was analyzed by focusing on the expression levels of genes encoding GTFs and stress ..
  33. Ding X, Yang Z, Han Y, Yu H. Fatty acid oxidation changes and the correlation with oxidative stress in different preeclampsia-like mouse models. PLoS ONE. 2014;9:e109554 pubmed publisher
    Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) expression is decreased in placenta of some cases of preeclampsia (PE) which may result in free fatty acid (FFA) increased...
  34. Yamamoto Y, Matsui N, Hiramatsu Y, Miyazaki Y, Nodera H, Izumi Y, et al. Mitochondrial trifunctional protein deficiency: an adult patient with similar progress to Charcot-Marie-Tooth disease. Rinsho Shinkeigaku. 2017;57:82-87 pubmed publisher
    ..MTP) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid beta-oxidation caused by HADHA or HADHB mutation...
  35. Nahid M, Ross S, Umiker B, Li H, Sugii S, Bari L. Development of porcine ficolin-alpha monoclonal and polyclonal antibodies for determining the binding capacity of multiple GlcNAc-binding proteins to bacterial danger components. Vet Immunol Immunopathol. 2016;170:13-9 pubmed publisher
    ..In this report, GlcNAc-binding protein (GBP) purified from porcine nonimmune serum was biochemically characterized as ficolin-α...
  36. Conti P, Ronconi G, Caraffa A, Lessiani G, Duraisamy K. IL-37 a New IL-1 Family Member Emerges as a Key Suppressor of Asthma Mediated by Mast Cells. Immunol Invest. 2017;46:239-250 pubmed publisher
    ..However, the precise mechanism(s), safety, and tolerability of IL-37 are unclear and still remain a mystery. GBP (Guanylate Binding Proteins); HMGB1 (High Mobility Group Box protein 1); NLRP (Nucleotide-like Receptor Pyrin ..
  37. Stefańska A, Gaffke L, Kaczorowska A, Plotka M, Dabrowski S, Kaczorowski T. Highly thermostable RadA protein from the archaeon Pyrococcus woesei enhances specificity of simplex and multiplex PCR assays. J Appl Genet. 2016;57:239-49 pubmed publisher
    ..Among applications tested the RadA protein proved to be useful in allelic discrimination assay of HADHA gene associated with long-chain 3-hydroxylacyl-CoA dehydrogenase deficiency that in infancy may lead to hypotonia, ..
  38. Lagerros Y, Brandt L, Hedberg J, Sundbom M, Boden R. Suicide, Self-harm, and Depression After Gastric Bypass Surgery: A Nationwide Cohort Study. Ann Surg. 2017;265:235-243 pubmed publisher
    The aim of this study was to examine risk of self-harm, hospitalization for depression and death by suicide after gastric bypass surgery (GBP).
  39. Satapathy A, Jain V, Ellard S, Flanagan S. Hyperinsulinemic Hypoglycemia of Infancy due to Novel HADH Mutation in Two Siblings. Indian Pediatr. 2016;53:912-913 pubmed
    ..Inactivating mutations in the genes ABCC8 and KCNJ11 are the commonest cause. Mutation in the HADH gene, which encodes the short-chain-L-3-hydroxyacyl-CoA dehydrogenase, is a rare cause...
  40. Amiri S, Haj Mirzaian A, Amini Khoei H, Razmi A, Shirzadian A, Rahimi Balaei M, et al. Protective effects of gabapentin against the seizure susceptibility and comorbid behavioral abnormalities in the early socially isolated mice. Eur J Pharmacol. 2017;797:106-114 pubmed publisher
    ..In this study, we aimed to investigate whether gabapentin (GBP) as an anti-epileptic drug is able to alleviate the seizure activity as well as comorbid behavioral abnormalities ..
  41. Ianuzzo C, Noble E, Hamilton N, Dabrowski B. Effects of streptozotocin diabetes, insulin treatment, and training on the diaphragm. J Appl Physiol Respir Environ Exerc Physiol. 1982;52:1471-5 pubmed
    ..beta-Oxidation enzyme 3-hydroxyacyl-CoA dehydrogenase (HADH) was 60% higher than normal in the diabetic diaphragm and returned to normal with insulin treatment...
  42. Ariotti N, Hall T, Rae J, Ferguson C, McMahon K, Martel N, et al. Modular Detection of GFP-Labeled Proteins for Rapid Screening by Electron Microscopy in Cells and Organisms. Dev Cell. 2015;35:513-25 pubmed publisher
    ..soybean ascorbate peroxidase (APEX) to any GFP-tagged protein of interest by engineering a GFP-binding peptide (GBP) directly to the APEX-tag...
  43. Wajner M, Amaral A. Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies. Biosci Rep. 2015;36:e00281 pubmed publisher
    ..common FAOD, including medium-chain acyl-CoA dehydrogenase (MCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and very long-chain acyl-CoA dehydrogenase (VLCAD) deficiencies...
  44. Orii K, Orii K, Souri M, Orii T, Kondo N, Hashimoto T, et al. Genes for the human mitochondrial trifunctional protein alpha- and beta-subunits are divergently transcribed from a common promoter region. J Biol Chem. 1999;274:8077-84 pubmed
    Human HADHA and HADHB genes encode the subunits of an enzyme complex, the trifunctional protein, involved in mitochondrial beta-oxidation of fatty acids. Both genes are located in the same region of chromosome 2p23...
  45. Komatsu Y, Sukegawa S, Yamashita M, Katsuda N, Tong B, Ohta T, et al. Identification of genes showing differential expression profile associated with growth rate in skeletal muscle tissue of Landrace weanling pig. J Genet. 2016;95:341-7 pubmed
    ..Three upregulated genes (EEF1A2, TSG101 and TTN) and six downregulated genes (ATP5B, ATP5C1, COQ3, HADHA, MYH1 and MYH7) in pig with genetic propensity for higher growth rate were identified by sequence analysis of 12 ..
  46. Schroder K, Escher S, Hoffmann Dörr S, Kühne R, Simetska N, Mangelsdorf I. Evaluation of route-to-route extrapolation factors based on assessment of repeated dose toxicity studies compiled in the database RepDose®. Toxicol Lett. 2016;261:32-40 pubmed publisher
    ..For the dermal route, our dataset was too small to allow general conclusions, but the results so far do suggest that the current ECHA guidance is conservative when assuming that dermal absorption is as high as oral absorption.
  47. Alles S, Bandet M, Eppler K, Noh M, Winship I, Baker G, et al. Acute anti-allodynic action of gabapentin in dorsal horn and primary somatosensory cortex: Correlation of behavioural and physiological data. Neuropharmacology. 2017;113:576-590 pubmed publisher
    ..Although first line treatments include the alpha-2-delta (α2δ)-ligands, pregabalin and gabapentin (GBP), the mechanism of their anti-allodynic action is poorly understood...
  48. Voloshanenko O, Schwartz U, Kranz D, Rauscher B, Linnebacher M, Augustin I, et al. β-catenin-independent regulation of Wnt target genes by RoR2 and ATF2/ATF4 in colon cancer cells. Sci Rep. 2018;8:3178 pubmed publisher
    ..Independent experiments confirmed several target genes, including PLOD2, HADH, LCOR and REEP1 as non-canonical target genes in various colon cancer cells...
  49. Verger E, Aron Wisnewsky J, Dao M, Kayser B, Oppert J, Bouillot J, et al. Micronutrient and Protein Deficiencies After Gastric Bypass and Sleeve Gastrectomy: a 1-year Follow-up. Obes Surg. 2016;26:785-96 pubmed publisher
    Roux-en-Y gastric bypass (GBP) and sleeve gastrectomy (SG) have increased dramatically, potentially increasing the prevalence of nutritional deficiencies...
  50. Strandqvist A, Haglind C, Zetterström R, Nemeth A, Von Dobeln U, Stenlid M, et al. Neuropsychological Development in Patients with Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase (LCHAD) Deficiency. JIMD Rep. 2016;28:75-84 pubmed
    ..Future studies are warranted to investigate whether newborn screening programs and early treatment may promote improved neuropsychological development and outcomes. ..
  51. Brinkmann M, Preuss T, Hollert H. Advancing In Vitro-In Vivo Extrapolations of Mechanism-Specific Toxicity Data Through Toxicokinetic Modeling. Adv Biochem Eng Biotechnol. 2017;157:293-317 pubmed publisher
    ..arising from these increased testing requirements, decision-makers, such as the European Chemicals Agency (ECHA), are committed to Russel and Burch's 3R principle (i.e...
  52. Boerrigter Eenling R, Alewijn M, Weesepoel Y, van Ruth S. New approaches towards discrimination of fresh/chilled and frozen/thawed chicken breasts by HADH activity determination: Customized slope fitting and chemometrics. Meat Sci. 2017;126:43-49 pubmed publisher
    ..of the fresh/thawed status of chicken meat is determined by measuring ?-hydroxyacyl-Coenzyme A-hydrogenase (HADH) activity present in meat intra-cellular liquids spectrophotometrically...
  53. Raghupathy V, Goel A, Thangaraj K, Eapen C, Balasubramanian K, Regi A, et al. Absence of G1528C mutation in long-chain 3-hydroxyacyl-CoA dehydrogenase in four Indian patients with pregnancy-related liver disease. Indian J Gastroenterol. 2014;33:387-9 pubmed publisher
  54. Visser E, Wegrzyn J, Steenkmap E, Myburg A, Naidoo S. Combined de novo and genome guided assembly and annotation of the Pinus patula juvenile shoot transcriptome. BMC Genomics. 2015;16:1057 pubmed publisher
    ..This was partially alleviated through release of the 22 Gbp Pinus taeda v1.01 genome sequence ( http://pinegenome.org/pinerefseq/ ) in 2014...
  55. Yasui Y, Hirakawa H, Oikawa T, Toyoshima M, Matsuzaki C, Ueno M, et al. Draft genome sequence of an inbred line of Chenopodium quinoa, an allotetraploid crop with great environmental adaptability and outstanding nutritional properties. DNA Res. 2016;23:535-546 pubmed
    ..The de novo genome assembly contained 25 k scaffolds consisting of 1 Gbp with N50 length of 86 kbp. Based on these data, we constructed the free-access Quinoa Genome DataBase (QGDB)...
  56. Tao R, Wang C, Zhang C, Li W, Zhou H, Chen H, et al. Characterization, Cytotoxicity, and Genotoxicity of TiO2 and Folate-Coupled Chitosan Nanoparticles Loading Polyprenol-Based Nanoemulsion. Biol Trace Elem Res. 2018;184:60-74 pubmed publisher
    The structure and bioactivity of Ginkgo biloba leaves polyprenol (GBP) are similar to that of dolichol which widely exists in human and mammalian organs. GBP possesses potential pharmacological activities against cancer...
  57. Martín Martín S, Rodríguez García A, Santos Beneit F, Franco Domínguez E, Sola Landa A, Martín J. Self-control of the PHO regulon: the PhoP-dependent protein PhoU controls negatively expression of genes of PHO regulon in Streptomyces coelicolor. J Antibiot (Tokyo). 2017;: pubmed publisher
    ..Whereas transcription of PhoU is entirely dependent on PhoP, expression of the downstream mtpA gene is only partially dependent on PhoP activation...
  58. Southey D, Pullinger D, Loggos S, Kumari N, Lengyel E, Morgan I, et al. Discharge of thoracic patients on portable digital suction: Is it cost-effective?. Asian Cardiovasc Thorac Ann. 2015;23:832-8 pubmed publisher
    ..Twenty were discharged home, equating to 772 bed-days saved (GBP 270,000 cost-saving). Clinic attendance totalled 162 visits (GBP 24,300 cost reimbursement for attendance)...
  59. Senniappan S, Sadeghizadeh A, Flanagan S, Ellard S, Hashemipour M, Hosseinzadeh M, et al. Genotype and phenotype correlations in Iranian patients with hyperinsulinaemic hypoglycaemia. BMC Res Notes. 2015;8:350 pubmed publisher
    ..Abnormalities in nine different genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A, UCP2 and HNF1A) have been reported in HH, the most common being ABCC8 and KCNJ11...
  60. Booth H, Khan O, Fildes A, Prevost A, Reddy M, Charlton J, et al. Changing Epidemiology of Bariatric Surgery in the UK: Cohort Study Using Primary Care Electronic Health Records. Obes Surg. 2016;26:1900-5 pubmed publisher
    ..procedures between 2002 and 2014, including laparoscopic adjustable gastric banding (LAGB), 1297; gastric bypass (GBP), 1265; and sleeve gastrectomy (SG), 477. Annual procedures increased from one in 2002 to a maximum of 525 in 2010...
  61. Makishi P, Thitthaweerat S, Sadr A, Shimada Y, Martins A, Tagami J, et al. Assessment of current adhesives in class I cavity: Nondestructive imaging using optical coherence tomography and microtensile bond strength. Dent Mater. 2015;31:e190-200 pubmed publisher
    ..Single Bond 2: ASB2), two-step self-etch adhesive (Clearfil SE Bond: CSEB), all-in-one adhesives (G-Bond Plus: GBP; Tri-S Bond Plus: TSBP), or no adhesive (Control), or bonded using low-shrinkage composite with its proper adhesive ..
  62. Wang C, Yuan J, Li W, Zhang H, Ye J. In Vivo and In Vitro Toxicity Evaluation of Polyprenols Extracted from Ginkgo biloba L. Leaves. Molecules. 2015;20:22257-71 pubmed publisher
    Polyprenols of Ginkgo biloba L. leaves (GBP) are a new type of lipid with 14-24 isoprenyl units, which in humans have strong bioactivity like the dolichols...
  63. Che L, Yang Z, Xu M, Xu S, Che L, Lin Y, et al. Maternal nutrition modulates fetal development by inducing placental efficiency changes in gilts. BMC Genomics. 2017;18:213 pubmed publisher
    ..In the Con group, these proteins were involved in lipid metabolism (HADHA, AACS, CAD), nutrient transport (GLUT, SLC27A1), and energy metabolism (NDUFV1, NDUFV2, ATP5C1)...
  64. Yang Z, Yamada J, Zhao Y, Strauss A, Ibdah J. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA. 2002;288:2163-6 pubmed
    ..Screening newborns for this mutation in pregnancies complicated by AFLP could allow early diagnosis and treatment in newborns and genetic counseling and prenatal diagnosis in subsequent pregnancies in affected families. ..
  65. Einset J, Collins A. Genome size and sensitivity to DNA damage by X-rays-plant comets tell the story. Mutagenesis. 2017;: pubmed publisher
    ..6 to 19.2 Gbp, showed that larger genomes are more sensitive to DNA damage by a relationship approximating the cube-root of the ..
  66. Hagenbuchner J, Scholl Buergi S, Karall D, Ausserlechner M. Very long-/ and long Chain-3-Hydroxy Acyl CoA Dehydrogenase Deficiency correlates with deregulation of the mitochondrial fusion/fission machinery. Sci Rep. 2018;8:3254 pubmed publisher
    ..By analyzing mitochondrial morphology we uncovered that mutations within the HADHA or the ACADVL gene not only affect fatty acid oxidation, but also cause significant changes in the DNM1L/MFN2 ..
  67. Yan W, Liu L, Qin W, Li C, Peng Z. Transcriptomic identification of chemoreceptor genes in the red palm weevil Rhynchophorus ferrugineus. Genet Mol Res. 2015;14:7469-80 pubmed publisher
    ..About 8.08 Gbp data were generated using a HiSeq platform and their assembly led to a total of 24,439 unigenes...
  68. Apitz J, Nishimura K, Schmied J, Wolf A, Hedtke B, van Wijk K, et al. Posttranslational Control of ALA Synthesis Includes GluTR Degradation by Clp Protease and Stabilization by GluTR-Binding Protein. Plant Physiol. 2016;170:2040-51 pubmed publisher
    ..caseinolytic protease (Clp) substrate adaptor ClpS1 and the ClpC1 chaperone as well as the GluTR-binding protein (GBP) interact with the N terminus of GluTR Loss-of function mutants of ClpR2 and ClpC1 proteins show increased GluTR ..
  69. Jones S, Taylor G, Chan S, Warren R, Hammond S, Bilobram S, et al. The Genome of the Beluga Whale (Delphinapterus leucas). Genes (Basel). 2017;8: pubmed publisher
    ..32 Gbp. To aid our understanding of the functional elements, transcriptome data was also derived from brain, duodenum, ..
  70. Iswarya A, Anjugam M, Vaseeharan B. Role of purified ?-1, 3 glucan binding protein (?-GBP) from Paratelphusa hydrodromus and their anti-inflammatory, antioxidant and antibiofilm properties. Fish Shellfish Immunol. 2017;68:54-64 pubmed publisher
    1, 3-glucan binding protein (?-GBP), a pattern recognition protein (PRP), plays a critical role in triggering the innate immune response by detecting ?-glucan found on the surface of microbes...
  71. López Montero N, Enninga J. Shigella Stays on the Move. Cell Host Microbe. 2017;22:432-433 pubmed publisher
    ..2017) report that cytosolic Shigella becomes trapped within a GBP coat that prevents bacterial cell-to-cell spread...
  72. Middleton B. The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA thiolase. Biochem Soc Trans. 1994;22:427-31 pubmed
  73. Paulino D, Warren R, Vandervalk B, Raymond A, Jackman S, Birol I. Sealer: a scalable gap-closing application for finishing draft genomes. BMC Bioinformatics. 2015;16:230 pubmed publisher
    ..We demonstrate how it scales to successfully close 50.8% and 13.8% of gaps in human (3 Gbp) and white spruce (20 Gbp) draft assemblies in under 30 and 27 h, respectively - a feat that is not possible with ..
  74. Liu H, Bai Q, Yao L, Zhang H, Xu H, Zhang S, et al. Highly efficient near ultraviolet organic light-emitting diode based on a meta-linked donor-acceptor molecule. Chem Sci. 2015;6:3797-3804 pubmed publisher
    ..a meta-linked donor-acceptor (D-A) structure between triphenylamine (TPA) and phenanthroimidazole (PPI), mTPA-PPI, was designed and synthesized...
  75. Weekes J, Morrison K, Mullen A, Wait R, Barton P, Dunn M. Hyperubiquitination of proteins in dilated cardiomyopathy. Proteomics. 2003;3:208-16 pubmed
    ..A number of these proteins were identified using affinity purification and matrix-assisted laser desorption/ionisation time-of-flight mass spectrometry. ..
  76. Hong H, Xu J, McGoogan J, Dong H, Xu G, Wu Z. Relationship between the use of gay mobile phone applications and HIV infection among men who have sex with men in Ningbo, China: a cross-sectional study. Int J STD AIDS. 2017;:956462417738468 pubmed publisher
    ..of the gay app users were younger than 19 years old, single, had high school education, were earning less than 115 GBP income per month, and identified themselves as homosexual...
  77. Ijlst L, Wanders R, Ushikubo S, Kamijo T, Hashimoto T. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein. Biochim Biophys Acta. 1994;1215:347-50 pubmed
    ..This mutation was not found in 55 different control subjects. This indicates a high frequency for this mutation in long-chain 3-hydroxyacyl-CoA dehydrogenase deficient patients. ..
  78. Kamijo T, Aoyama T, Komiyama A, Hashimoto T. Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein. Biochem Biophys Res Commun. 1994;199:818-25 pubmed
    ..Expression of this cDNA in mammalian cells yielded a polypeptide with the long-chain 3-ketoacyl-CoA thiolase activity. ..
  79. Ibdah J, Tein I, Dionisi Vici C, Bennett M, Ijlst L, Gibson B, et al. Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation. J Clin Invest. 1998;102:1193-9 pubmed
  80. Griffin A, Strauss A, Bennett M, Ernst L. Mutations in long-chain 3-hydroxyacyl coenzyme a dehydrogenase are associated with placental maternal floor infarction/massive perivillous fibrin deposition. Pediatr Dev Pathol. 2012;15:368-74 pubmed publisher
    ..The placenta of an 8-month-old child diagnosed with long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) deficiency reportedly showed MFI, but no further evidence of a direct association between MFI/MPVFD and LCHAD ..
  81. De Groot R, Brekelmans P, Desel H, de Vries I. New legal requirements for submission of product information to poisons centres in EU member states. Clin Toxicol (Phila). 2018;56:1-6 pubmed publisher
    ..together with guidance documents are expected to be made available by the end of 2017 by the European Chemicals Agency (ECHA). Guidance on interpretation of legal text and ECHA helpdesk support are planned to be ready at the end of 2018.
  82. Araya S, Martins A, Junqueira N, Costa A, Faleiro F, Ferreira M. Microsatellite marker development by partial sequencing of the sour passion fruit genome (Passiflora edulis Sims). BMC Genomics. 2017;18:549 pubmed publisher
    ..edulis genome in order to develop hundreds of new microsatellite markers. A total of 14.11 Gbp of Illumina paired-end sequence reads were analyzed to detect simple sequence repeat sites in the sour passion ..
  83. Praefcke G. Regulation of innate immune functions by guanylate-binding proteins. Int J Med Microbiol. 2018;308:237-245 pubmed publisher
    Guanylate-binding proteins (GBP) are a family of dynamin-related large GTPases which are expressed in response to interferons and other pro-inflammatory cytokines...
  84. Lefebvre C, Khartabil H, Boisson J, Contreras García J, Piquemal J, Henon E. Independent Gradient Model: a new approach for probing strong and weak interactions in molecules from wave function calculations. Chemphyschem. 2017;: pubmed publisher
    ..To this end, we formulate the Gradient-Based Partitioning (GBP) to assess the contribution of each orbital to the total ED gradient...
  85. Aoyama T, Wakui K, Orii K, Hashimoto T, Fukushima Y. Fluorescence in situ hybridization mapping of the alpha and beta subunits (HADHA and HADHB) of human mitochondrial fatty acid beta-oxidation multienzyme complex to 2p23 and their evolution. Cytogenet Cell Genet. 1997;79:221-4 pubmed
    ..The alpha and beta subunits (HADHA and HADHB) are members of the enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase and 3-ketoacyl-CoA thiolase ..
  86. Spiekerkoetter U, Khuchua Z, Yue Z, Bennett M, Strauss A. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover. Pediatr Res. 2004;55:190-6 pubmed
    ..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. ..
  87. Krieger J, Taylor P, Gajadhar A, Guha A, Moran M, McGlade C. Identification and selected reaction monitoring (SRM) quantification of endocytosis factors associated with Numb. Mol Cell Proteomics. 2013;12:499-514 pubmed publisher
    ..Hence, quantitative mass spectrometric analysis of Numb protein-protein interactions has provided new insights into the assembly and regulation of protein complexes important in development and cancer. ..
  88. Kakumani P, Shanmugam R, Kaur I, Malhotra P, Mukherjee S, Bhatnagar R. Association of HADHA with human RNA silencing machinery. Biochem Biophys Res Commun. 2015;466:481-5 pubmed publisher
    ..In the present study, we investigated HADHA, a new component in the human RNA silencing machinery...
  89. Anjugam M, Vaseeharan B, Iswarya A, Amala M, Govindarajan M, Alharbi N, et al. A study on ?-glucan binding protein (?-GBP) and its involvement in phenoloxidase cascade in Indian white shrimp Fenneropenaeus indicus. Mol Immunol. 2017;92:1-11 pubmed publisher
    ..molecule ?-1, 3 glucan binding protein from the heamolymph of the Indian white shrimp, Fenneropenaeus indicus (Fi?-GBP). The purified Fi?-GBP had 95kDa molecular weight in SDS-PAGE analysis...
    Chronic complications observed in patients with long-chain 3-hydroxyacylCoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency may be mediated by the accumulation of 3-hydroxy fatty acids or 3-hydroxyacylcarnitines...
  91. Li Y, Franklin S, Zhang M, Vondriska T. Highly efficient purification of protein complexes from mammalian cells using a novel streptavidin-binding peptide and hexahistidine tandem tag system: application to Bruton's tyrosine kinase. Protein Sci. 2011;20:140-9 pubmed publisher
  92. Kiema T, Harijan R, Strozyk M, Fukao T, Alexson S, Wierenga R. The crystal structure of human mitochondrial 3-ketoacyl-CoA thiolase (T1): insight into the reaction mechanism of its thiolase and thioesterase activities. Acta Crystallogr D Biol Crystallogr. 2014;70:3212-25 pubmed publisher
    ..The fastest rate is observed for the hydrolysis of butyryl-CoA. It is also shown that T1 has significant biosynthetic thiolase activity, which is predicted to be of physiological importance. ..