| Effects of diet and physical activity interventions on weight loss and cardiometabolic risk factors in severely obese adults: a randomized trial BH Goodpaster, JP DeLany, AD Otto, L Kuller, J Vockley, JE South-Paul, ... Jama 304 (16), 1795-1802, 2010 | 766 | 2010 |
| Increased levels of plasma acylcarnitines in obesity and type 2 diabetes and identification of a marker of glucolipotoxicity SJ Mihalik, BH Goodpaster, DE Kelley, DH Chace, J Vockley, FGS Toledo, ... Obesity 18 (9), 1695-1700, 2010 | 662 | 2010 |
| Phenylalanine hydroxylase deficiency: diagnosis and management guideline J Vockley, HC Andersson, KM Antshel, NE Braverman, BK Burton, ... Genetics in medicine 16 (2), 188-200, 2014 | 640 | 2014 |
| Phenylketonuria Scientific Review Conference: state of the science and future research needs KM Camp, MA Parisi, PB Acosta, GT Berry, DA Bilder, N Blau, ... Molecular genetics and metabolism 112 (2), 87-122, 2014 | 306 | 2014 |
| Metabolomic profiling of fatty acid and amino acid metabolism in youth with obesity and type 2 diabetes: evidence for enhanced mitochondrial oxidation SJ Mihalik, SF Michaliszyn, J De Las Heras, F Bacha, SJ Lee, DH Chace, ... Diabetes care 35 (3), 605-611, 2012 | 269 | 2012 |
| Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals crucial roles for fatty acid oxidation DM Kurtz, P Rinaldo, WJ Rhead, L Tian, DS Millington, J Vockley, ... Proceedings of the National Academy of Sciences 95 (26), 15592-15597, 1998 | 265 | 1998 |
| Isovaleric acidemia: new aspects of genetic and phenotypic heterogeneity J Vockley, R Ensenauer American journal of medical genetics part C: seminars in medical genetics …, 2006 | 260 | 2006 |
| Recommendations for the nutrition management of phenylalanine hydroxylase deficiency RH Singh, F Rohr, D Frazier, A Cunningham, S Mofidi, B Ogata, PL Splett, ... Genetics in Medicine 16 (2), 121-131, 2014 | 251 | 2014 |
| Synergistic heterozygosity: disease resulting from multiple partial defects in one or more metabolic pathways J Vockley, P Rinaldo, MJ Bennett, D Matern, GD Vladutiu Molecular genetics and metabolism 71 (1-2), 10-18, 2000 | 240 | 2000 |
| A proposed nosology of inborn errors of metabolism CR Ferreira, CDM van Karnebeek, J Vockley, N Blau Genetics in medicine 21 (1), 102-106, 2019 | 235 | 2019 |
| Efficacy of sapropterin dihydrochloride in increasing phenylalanine tolerance in children with phenylketonuria: a phase III, randomized, double-blind, placebo-controlled study FK Trefz, BK Burton, N Longo, MMP Casanova, DJ Gruskin, A Dorenbaum, ... The Journal of pediatrics 154 (5), 700-707. e1, 2009 | 233 | 2009 |
| The genetic landscape and epidemiology of phenylketonuria A Hillert, Y Anikster, A Belanger-Quintana, A Burlina, BK Burton, ... The American Journal of Human Genetics 107 (2), 234-250, 2020 | 210 | 2020 |
| Molecular cloning and nucleotide sequence of cDNAs encoding the precursors of rat long chain acyl-coenzyme A, short chain acyl-coenzyme A, and isovaleryl-coenzyme A … Y Matsubara, Y Indo, E Naito, H Ozasa, R Glassberg, J Vockley, Y Ikeda, ... Journal of Biological Chemistry 264 (27), 16321-16331, 1989 | 210 | 1989 |
| A common mutation is associated with a mild, potentially asymptomatic phenotype in patients with isovaleric acidemia diagnosed by newborn screening R Ensenauer, J Vockley, JM Willard, JC Huey, JO Sass, SD Edland, ... The American Journal of Human Genetics 75 (6), 1136-1142, 2004 | 192 | 2004 |
| Evidence for physical association of mitochondrial fatty acid oxidation and oxidative phosphorylation complexes Y Wang, AW Mohsen, SJ Mihalik, ES Goetzman, J Vockley Journal of biological chemistry 285 (39), 29834-29841, 2010 | 187 | 2010 |
| Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death A Mathur, HF Sims, D Gopalakrishnan, B Gibson, P Rinaldo, J Vockley, ... Circulation 99 (10), 1337-1343, 1999 | 187 | 1999 |
| Barriers to the successful treatment of liver disease by hepatocyte transplantation KA Soltys, A Soto-Gutiérrez, M Nagaya, KM Baskin, M Deutsch, R Ito, ... Journal of hepatology 53 (4), 769-774, 2010 | 186 | 2010 |
| Single-dose, subcutaneous recombinant phenylalanine ammonia lyase conjugated with polyethylene glycol in adult patients with phenylketonuria: an open-label, multicentre, phase … N Longo, CO Harding, BK Burton, DK Grange, J Vockley, M Wasserstein, ... The Lancet 384 (9937), 37-44, 2014 | 184 | 2014 |
| Sirtuin 3 (SIRT3) protein regulates long-chain acyl-CoA dehydrogenase by deacetylating conserved lysines near the active site SS Bharathi, Y Zhang, AW Mohsen, R Uppala, M Balasubramani, ... Journal of Biological Chemistry 288 (47), 33837-33847, 2013 | 183 | 2013 |
| Liver transplantation for classical maple syrup urine disease: long-term follow-up in 37 patients and comparative United Network for Organ Sharing experience GV Mazariegos, DH Morton, R Sindhi, K Soltys, N Nayyar, G Bond, ... The Journal of pediatrics 160 (1), 116-121. e1, 2012 | 182 | 2012 |
