PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 33725513-4 2021 Proteomic data that was corroborated by antibody-based detection, indicated reduced levels of VLCAD and TFP protein in cells with VLCAD and TFP mutations respectively, which in part accounted for the diminished fatty acid oxidation capacity. Fatty Acids 211-221 acyl-CoA dehydrogenase very long chain Homo sapiens 94-99 33725513-4 2021 Proteomic data that was corroborated by antibody-based detection, indicated reduced levels of VLCAD and TFP protein in cells with VLCAD and TFP mutations respectively, which in part accounted for the diminished fatty acid oxidation capacity. Fatty Acids 211-221 acyl-CoA dehydrogenase very long chain Homo sapiens 130-135 30401918-1 2019 Mitochondrial fatty acid oxidation disorders (FAODs) are caused by defects in beta-oxidation enzymes, including very long-chain acyl-CoA dehydrogenase (VLCAD), trifunctional protein (TFP), carnitine palmitoyltransferase-2 (CPT2), carnitine-acylcarnitine translocase (CACT) and others. Fatty Acids 14-24 acyl-CoA dehydrogenase very long chain Homo sapiens 112-150 33597881-1 2020 Patients with a deficiency in very long-chain acyl-CoA dehydrogenase (VLCAD), an enzyme that is involved in the mitochondrial beta-oxidation of long-chain fatty acids, are at risk for developing cardiac arrhythmias. Fatty Acids 155-166 acyl-CoA dehydrogenase very long chain Homo sapiens 70-75 32669490-1 2020 Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a genetic disorder of fatty acid beta oxidation that is caused by a defect in ACADVL, which encodes VLCAD. Fatty Acids 83-93 acyl-CoA dehydrogenase very long chain Homo sapiens 139-145 32669490-1 2020 Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a genetic disorder of fatty acid beta oxidation that is caused by a defect in ACADVL, which encodes VLCAD. Fatty Acids 83-93 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 30401918-1 2019 Mitochondrial fatty acid oxidation disorders (FAODs) are caused by defects in beta-oxidation enzymes, including very long-chain acyl-CoA dehydrogenase (VLCAD), trifunctional protein (TFP), carnitine palmitoyltransferase-2 (CPT2), carnitine-acylcarnitine translocase (CACT) and others. Fatty Acids 14-24 acyl-CoA dehydrogenase very long chain Homo sapiens 152-157 27644403-1 2016 Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first reaction in the mitochondrial fatty acid beta-oxidation pathway. Fatty Acids 97-107 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 29459657-2 2018 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 ratio leading to the significant accumulation of truncated and punctate mitochondria in contrast to network-like mitochondrial morphology in controls. Fatty Acids 118-128 acyl-CoA dehydrogenase very long chain Homo sapiens 90-96 29499131-3 2018 We report that the BCL-2 homology 3 (BH3) alpha helix of MCL-1 can directly engage very long-chain acyl-CoA dehydrogenase (VLCAD), a key enzyme of the mitochondrial fatty acid beta-oxidation (FAO) pathway. Fatty Acids 165-175 acyl-CoA dehydrogenase very long chain Homo sapiens 83-121 29499131-3 2018 We report that the BCL-2 homology 3 (BH3) alpha helix of MCL-1 can directly engage very long-chain acyl-CoA dehydrogenase (VLCAD), a key enzyme of the mitochondrial fatty acid beta-oxidation (FAO) pathway. Fatty Acids 165-175 acyl-CoA dehydrogenase very long chain Homo sapiens 123-128 28980192-1 2018 BACKGROUND: Very long chain acyl CoA dehydrogenase (VLCAD) deficiency (OMIM#201475) is an autosomal recessive disorder of fatty acid beta oxidation caused by defect in the ACADVL. Fatty Acids 122-132 acyl-CoA dehydrogenase very long chain Homo sapiens 172-178 27076444-2 2016 In a GeneFishing experiment using human preeclamptic placenta, expression of acyl-coenzyme A dehydrogenase very long chain (ACADVL), which is involved in fatty acid beta-oxidation (FAO), was detected. Fatty Acids 154-164 acyl-CoA dehydrogenase very long chain Homo sapiens 77-122 27076444-2 2016 In a GeneFishing experiment using human preeclamptic placenta, expression of acyl-coenzyme A dehydrogenase very long chain (ACADVL), which is involved in fatty acid beta-oxidation (FAO), was detected. Fatty Acids 154-164 acyl-CoA dehydrogenase very long chain Homo sapiens 124-130 27644403-1 2016 Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first reaction in the mitochondrial fatty acid beta-oxidation pathway. Fatty Acids 97-107 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 26147000-11 2015 The verification of different proteins identified in our previous proteomic study shows that the enzymes of anaerobic catabolism were up-regulated (PFKP and LDHA), and the enzymes of aerobic oxidation and fatty acid oxidation were down-regulated (CS, DLST, PGD, ACSL4, ACADVL and HADHA) in RA patients. Fatty Acids 205-215 acyl-CoA dehydrogenase very long chain Homo sapiens 269-275 26051273-8 2015 Additionally, nickel down-regulated the protein levels of the key fatty acid oxidation enzyme very long-chain acyl-CoA dehydrogenase (VLCAD) in a dose-dependent fashion. Fatty Acids 66-76 acyl-CoA dehydrogenase very long chain Homo sapiens 94-132 26051273-8 2015 Additionally, nickel down-regulated the protein levels of the key fatty acid oxidation enzyme very long-chain acyl-CoA dehydrogenase (VLCAD) in a dose-dependent fashion. Fatty Acids 66-76 acyl-CoA dehydrogenase very long chain Homo sapiens 134-139 25811481-2 2015 Here, we demonstrate that SIRT3 and SIRT5 both target human very long-chain acyl-CoA dehydrogenase (VLCAD), a key fatty acid oxidation enzyme. Fatty Acids 114-124 acyl-CoA dehydrogenase very long chain Homo sapiens 60-98 25737446-3 2015 Treatment of VLCAD-deficient fibroblasts, which express distinct mutant VLCAD protein and exhibit deficient fatty acid beta-oxidation, with S-nitroso-N-acetylcysteine induced site-specific S-nitrosylation of VLCAD mutants at cysteine residue 237. Fatty Acids 108-118 acyl-CoA dehydrogenase very long chain Homo sapiens 13-18 25811481-2 2015 Here, we demonstrate that SIRT3 and SIRT5 both target human very long-chain acyl-CoA dehydrogenase (VLCAD), a key fatty acid oxidation enzyme. Fatty Acids 114-124 acyl-CoA dehydrogenase very long chain Homo sapiens 100-105 25811481-9 2015 Thus, SIRT3 and SIRT5 promote fatty acid oxidation by converging upon VLCAD to promote its activity and membrane localization. Fatty Acids 30-40 acyl-CoA dehydrogenase very long chain Homo sapiens 70-75 23185754-6 2012 Of them, genes correlated to lipid metabolism included CRLS1, LRP11, FUT9, GPCPD1, PIGL, SULT1A4, B3GNT1, ST8SIA4, and ACADVL, mainly involved in the metabolic processes of fatty acid, cholesterol, phospholipids, and glycolipid. Fatty Acids 173-183 acyl-CoA dehydrogenase very long chain Homo sapiens 119-125 25843429-3 2015 VLCAD is one of four acyl-CoA dehydrogenases with different-chain length specificity and catalyzes the initial step in mitochondrial beta-oxidation of fatty acids. Fatty Acids 151-162 acyl-CoA dehydrogenase very long chain Homo sapiens 0-5 19156135-2 2009 VLCAD is a mitochondrial enzyme involved in fatty acid beta-oxidation, a key step in energy production during times of fasting or stress. Fatty Acids 44-54 acyl-CoA dehydrogenase very long chain Homo sapiens 0-5 19649258-1 2009 BACKGROUND: Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respectively) deficiency, using acylcarnitine profiling with tandem mass spectrometry, has increased the number of patients with fatty acid oxidation disorders due to the identification of additional milder, and so far silent, phenotypes. Fatty Acids 231-241 acyl-CoA dehydrogenase very long chain Homo sapiens 95-100 22093833-5 2011 The peptide sequence was similar to the sequence of amino acids 644-655 of C-terminal extra-polypeptide of very-long-chain acyl-CoA dehydrogenase (VLCAD), which is 1 of 4 flavoproteins that catalyzing the initial step of the mitochondrial fatty acid beta-oxidation, implying a close relationship between SOCS3 and VLCAD. Fatty Acids 239-249 acyl-CoA dehydrogenase very long chain Homo sapiens 107-145 22093833-5 2011 The peptide sequence was similar to the sequence of amino acids 644-655 of C-terminal extra-polypeptide of very-long-chain acyl-CoA dehydrogenase (VLCAD), which is 1 of 4 flavoproteins that catalyzing the initial step of the mitochondrial fatty acid beta-oxidation, implying a close relationship between SOCS3 and VLCAD. Fatty Acids 239-249 acyl-CoA dehydrogenase very long chain Homo sapiens 147-152 22093833-7 2011 Animal experimentation demonstrated that VLCAD is functionally involved in SOCS3 binding and thus, SOCS3 play an important role in the regulation of fatty acid beta-oxidation. Fatty Acids 149-159 acyl-CoA dehydrogenase very long chain Homo sapiens 41-46 20816094-6 2010 This strongly contrasts with its evolutionary ancestor VLCAD, which we show is not required for complex I assembly and clearly plays a role in fatty acid oxidation. Fatty Acids 143-153 acyl-CoA dehydrogenase very long chain Homo sapiens 55-60 20480395-6 2010 In the current study, we use the fatty acid oxidation disorder very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the second most common fatty acid oxidation disorder detected by expanded newborn screening, to demonstrate accurate and fast diagnostic evaluation of the ACADVL gene utilizing DNA extracted from the newborn screening dried blood spot and high resolution melt (HRM) profiling. Fatty Acids 33-43 acyl-CoA dehydrogenase very long chain Homo sapiens 278-284 19889959-9 2010 Furthermore, a phosphorylation-negative mutant (S586A) VLCAD shows reduced electron transfer activity and a strong dominant-negative effect on fatty acid beta-oxidation. Fatty Acids 143-153 acyl-CoA dehydrogenase very long chain Homo sapiens 55-60 19535822-10 2009 This review summarizes recent observations on disorders associated with fatty-acid oxidation: deficiencies of beta-oxidation enzymes, namely VLCAD, TFP and LCHAD, MCAD, MCKAT, M/SCHAD, and SCAD, and deficiencies of the enzymes TCP I, CT, and CPT II of the carnitine cycle. Fatty Acids 72-82 acyl-CoA dehydrogenase very long chain Homo sapiens 141-146 10700700-4 2000 Newborn screening by tandem mass spectrometry will detect disorders of fatty acid oxidation such as VLCAD and allow early and preventive treatment. Fatty Acids 71-81 acyl-CoA dehydrogenase very long chain Homo sapiens 100-105 17374501-1 2007 Very long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first enzymatic step in the mitochondrial beta-oxidation of fatty acids 14-20 carbons in length. Fatty Acids 121-132 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 17374501-1 2007 Very long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first enzymatic step in the mitochondrial beta-oxidation of fatty acids 14-20 carbons in length. Fatty Acids 121-132 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 11524729-5 2001 In the present review we summarize current knowledge regarding genotype-phenotype relationships in three disorders of mitochondrial fatty acid oxidation: very-long chain acyl-CoA dehydrogenase (VLCAD, also ACADVL), medium-chain acyl-CoA dehydrogenase (MCAD, also ACADM), and short-chain acyl-CoA dehydrogenase (SCAD, also ACADS) deficiencies. Fatty Acids 132-142 acyl-CoA dehydrogenase very long chain Homo sapiens 154-192 11524729-5 2001 In the present review we summarize current knowledge regarding genotype-phenotype relationships in three disorders of mitochondrial fatty acid oxidation: very-long chain acyl-CoA dehydrogenase (VLCAD, also ACADVL), medium-chain acyl-CoA dehydrogenase (MCAD, also ACADM), and short-chain acyl-CoA dehydrogenase (SCAD, also ACADS) deficiencies. Fatty Acids 132-142 acyl-CoA dehydrogenase very long chain Homo sapiens 194-199 10607478-1 1999 Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of a family of nuclear-encoded enzymes that catalyze the initial step in mitochondrial fatty acid beta-oxidation (FAO). Fatty Acids 141-151 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 10607478-1 1999 Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of a family of nuclear-encoded enzymes that catalyze the initial step in mitochondrial fatty acid beta-oxidation (FAO). Fatty Acids 141-151 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 9973285-1 1999 Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta-oxidation. Fatty Acids 105-115 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 10077518-2 1999 Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first step in the beta-oxidation spiral of fatty acid metabolism, the crucial pathway for cardiac energy production. Fatty Acids 104-114 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 10077518-2 1999 Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first step in the beta-oxidation spiral of fatty acid metabolism, the crucial pathway for cardiac energy production. Fatty Acids 104-114 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 9973285-1 1999 Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta-oxidation. Fatty Acids 105-115 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 8845838-1 1996 Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are all nuclear encoded mitochondrial flavoproteins catalyzing the initial step in fatty acid beta-oxidation. Fatty Acids 204-214 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 9498103-1 1998 Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an enzyme catalyzing the dehydrogenation of long-chain fatty acids in the first step of mitochondrial fatty acid oxidation. Fatty Acids 105-115 acyl-CoA dehydrogenase very long chain Homo sapiens 0-38 9498103-1 1998 Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an enzyme catalyzing the dehydrogenation of long-chain fatty acids in the first step of mitochondrial fatty acid oxidation. Fatty Acids 105-115 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 8845838-1 1996 Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are all nuclear encoded mitochondrial flavoproteins catalyzing the initial step in fatty acid beta-oxidation. Fatty Acids 204-214 acyl-CoA dehydrogenase very long chain Homo sapiens 40-45 34245263-4 2021 RESULTS: IUGR fetuses exhibited an upregulation of key genes associated with fatty acid breakdown and beta-oxidation (Acadvl, Acadl, Acaa2), and mitochondrial carnitine shuttle (Cpt1a, Cpt2), instigating a metabolic gene reprogramming in the heart. Fatty Acids 77-87 acyl-CoA dehydrogenase very long chain Homo sapiens 118-124 8650121-9 1996 The successful prenatal diagnosis of VLCAD and MCAD deficiencies using in vitro probes of fatty acid oxidation in fibroblasts suggests that this approach can potentially recognize many mitochondrial fatty acid oxidation defects even if no prior diagnosis is determined in the family at risk. Fatty Acids 90-100 acyl-CoA dehydrogenase very long chain Homo sapiens 37-42 8650121-9 1996 The successful prenatal diagnosis of VLCAD and MCAD deficiencies using in vitro probes of fatty acid oxidation in fibroblasts suggests that this approach can potentially recognize many mitochondrial fatty acid oxidation defects even if no prior diagnosis is determined in the family at risk. Fatty Acids 199-209 acyl-CoA dehydrogenase very long chain Homo sapiens 37-42 34437764-1 2022 Inborn errors of mitochondrial fatty acid oxidation (FAO), such as medium-chain acyl-CoA dehydrogenase deficiency (MCAD) and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) affects cellular function and whole-body metabolism. Fatty Acids 31-41 acyl-CoA dehydrogenase very long chain Homo sapiens 176-181 34740033-11 2021 Expression of genes VLCAD and DGAT1, involved in fatty acid oxidation as well as DGAT1 in TAG synthesis, were significantly elevated after each cycle, whereas expression of genes ELOVL2 and FADS2, involved in fatty acid elongation and desaturation were significantly lower at D9 compared to D2 and D0 (P < 0.03). Fatty Acids 49-59 acyl-CoA dehydrogenase very long chain Homo sapiens 20-25 35001339-1 2022 PURPOSE: Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an essential mediator in fatty acid metabolism. Fatty Acids 84-94 acyl-CoA dehydrogenase very long chain Homo sapiens 9-47 34465376-1 2021 BACKGROUND: Very long-chain acyl-coenzyme-A dehydrogenase deficiency is a rare, severe life-threatening metabolic disorder of mitochondrial fatty acid oxidation, caused by mutations in ACADVL gene. Fatty Acids 140-150 acyl-CoA dehydrogenase very long chain Homo sapiens 185-191 35001339-1 2022 PURPOSE: Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an essential mediator in fatty acid metabolism. Fatty Acids 84-94 acyl-CoA dehydrogenase very long chain Homo sapiens 49-54