Peter W Stacpoole

Summary

Affiliation: University of Florida
Country: USA

Publications

  1. pmc Design and implementation of the first randomized controlled trial of coenzyme CoQ₁₀ in children with primary mitochondrial diseases
    Peter W Stacpoole
    University of Florida, USA
    Mitochondrion 12:623-9. 2012
  2. doi request reprint The pyruvate dehydrogenase complex as a therapeutic target for age-related diseases
    Peter W Stacpoole
    Departments of Medicine Division of Endocrinology and Metabolism and Biochemistry and Molecular Biology, College of Medicine, University of Florida, Gainesville, FL 32611, USA
    Aging Cell 11:371-7. 2012
  3. pmc The dichloroacetate dilemma: environmental hazard versus therapeutic goldmine--both or neither?
    Peter W Stacpoole
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, Florida 32610, USA
    Environ Health Perspect 119:155-8. 2011
  4. pmc Why are there no proven therapies for genetic mitochondrial diseases?
    Peter W Stacpoole
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32610, USA
    Mitochondrion 11:679-85. 2011
  5. ncbi request reprint Homocysteine synthesis is elevated but total remethylation is unchanged by the methylenetetrahydrofolate reductase 677C->T polymorphism and by dietary folate restriction in young women
    Steven R Davis
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville 32611 0370, USA
    J Nutr 135:1045-50. 2005
  6. pmc Moderate vitamin B-6 restriction does not alter postprandial methionine cycle rates of remethylation, transmethylation, and total transsulfuration but increases the fractional synthesis rate of cystathionine in healthy young men and women
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville, FL 32611 0370, USA
    J Nutr 141:835-42. 2011
  7. pmc Evaluation of long-term treatment of children with congenital lactic acidosis with dichloroacetate
    Peter W Stacpoole
    Department of Medicine, General Clinical Research Center, JHMHC, Gainesville, FL 32610 0226, USA
    Pediatrics 121:e1223-8. 2008
  8. pmc Vitamin B-6 restriction tends to reduce the red blood cell glutathione synthesis rate without affecting red blood cell or plasma glutathione concentrations in healthy men and women
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville, FL 32611 0370, USA
    Am J Clin Nutr 90:336-43. 2009
  9. pmc A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate
    Zongchao Han
    Department of Pediatrics, Division of Cellular and Molecular Therapy, University of Florida College of Medicine, Gainesville, FL, USA
    Mol Genet Metab 93:381-7. 2008
  10. pmc Chloride and other anions inhibit dichloroacetate-induced inactivation of human liver GSTZ1 in a haplotype-dependent manner
    Guo Zhong
    Department of Medicinal Chemistry, University of Florida, Gainesville, FL 32610 0485, United States
    Chem Biol Interact 215:33-9. 2014

Collaborators

Detail Information

Publications57

  1. pmc Design and implementation of the first randomized controlled trial of coenzyme CoQ₁₀ in children with primary mitochondrial diseases
    Peter W Stacpoole
    University of Florida, USA
    Mitochondrion 12:623-9. 2012
    ..These attitudes represent significant barriers to the ethical and scientific evaluation--and ultimate approval--of nutritional and pharmacological therapies for patients with life-threatening inborn errors of energy metabolism...
  2. doi request reprint The pyruvate dehydrogenase complex as a therapeutic target for age-related diseases
    Peter W Stacpoole
    Departments of Medicine Division of Endocrinology and Metabolism and Biochemistry and Molecular Biology, College of Medicine, University of Florida, Gainesville, FL 32611, USA
    Aging Cell 11:371-7. 2012
    ....
  3. pmc The dichloroacetate dilemma: environmental hazard versus therapeutic goldmine--both or neither?
    Peter W Stacpoole
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, Florida 32610, USA
    Environ Health Perspect 119:155-8. 2011
    ..Thus, DCA holds an almost unique position at the interface between environmental science and allopathic medicine...
  4. pmc Why are there no proven therapies for genetic mitochondrial diseases?
    Peter W Stacpoole
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32610, USA
    Mitochondrion 11:679-85. 2011
    ....
  5. ncbi request reprint Homocysteine synthesis is elevated but total remethylation is unchanged by the methylenetetrahydrofolate reductase 677C->T polymorphism and by dietary folate restriction in young women
    Steven R Davis
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville 32611 0370, USA
    J Nutr 135:1045-50. 2005
    ..In conclusion, elevated homocysteine synthesis is a cause of mild hyperhomocysteinemia in women with marginal folate status, particularly those with the MTHFR 677 T/T genotype...
  6. pmc Moderate vitamin B-6 restriction does not alter postprandial methionine cycle rates of remethylation, transmethylation, and total transsulfuration but increases the fractional synthesis rate of cystathionine in healthy young men and women
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville, FL 32611 0370, USA
    J Nutr 141:835-42. 2011
    ..Interrelationships among plasma concentrations of glycine and cystathionine and kinetic results suggest that individual variability occurs in normal postprandial 1-carbon metabolism and in the response to vitamin B-6 restriction...
  7. pmc Evaluation of long-term treatment of children with congenital lactic acidosis with dichloroacetate
    Peter W Stacpoole
    Department of Medicine, General Clinical Research Center, JHMHC, Gainesville, FL 32610 0226, USA
    Pediatrics 121:e1223-8. 2008
    ..The purpose of this research was to report results on long-term administration of dichloroacetate in 36 children with congenital lactic acidosis who participated previously in a controlled trial of this drug...
  8. pmc Vitamin B-6 restriction tends to reduce the red blood cell glutathione synthesis rate without affecting red blood cell or plasma glutathione concentrations in healthy men and women
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville, FL 32611 0370, USA
    Am J Clin Nutr 90:336-43. 2009
    ..Despite this obligatory role of PLP, previous studies from this laboratory showed that vitamin B-6 deficiency caused elevated glutathione concentrations in rat liver and human plasma...
  9. pmc A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate
    Zongchao Han
    Department of Pediatrics, Division of Cellular and Molecular Therapy, University of Florida College of Medicine, Gainesville, FL, USA
    Mol Genet Metab 93:381-7. 2008
    ..These data suggest that a combined gene delivery and pharmacological approach may hold promise for the treatment of PDH deficiency...
  10. pmc Chloride and other anions inhibit dichloroacetate-induced inactivation of human liver GSTZ1 in a haplotype-dependent manner
    Guo Zhong
    Department of Medicinal Chemistry, University of Florida, Gainesville, FL 32610 0485, United States
    Chem Biol Interact 215:33-9. 2014
    ..6±1.1mM (mean±S.D., n=3). Because the in vivo half-life of DCA is determined by the fraction of active GSTZ1 in the liver, identifying factors that regulate GSTZ1 activity is important in determining appropriate DCA dosing in humans. ..
  11. pmc Moderate dietary vitamin B-6 restriction raises plasma glycine and cystathionine concentrations while minimally affecting the rates of glycine turnover and glycine cleavage in healthy men and women
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville, FL 32611, USA
    J Nutr 139:452-60. 2009
    ..We conclude that the in vivo GCS and SHMT reactions are quite resilient to the effects of marginal vitamin B-6 deficiency, presumably through a compensatory effect of increasing substrate concentration...
  12. ncbi request reprint A GC-MS/MS method for the quantitative analysis of low levels of the tyrosine metabolites maleylacetone, succinylacetone, and the tyrosine metabolism inhibitor dichloroacetate in biological fluids and tissues
    Melissa D Zolodz
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida, Gainesville, FL 32610, USA
    J Chromatogr B Analyt Technol Biomed Life Sci 837:125-32. 2006
    ..To increase sensitivity, analytes were extracted from liver, urine, plasma and cultured nerve cells before and after dosing with DCA, derivatized to their pentafluorobenzyl esters, and analyzed via GC-MS/MS...
  13. pmc The heme precursor delta-aminolevulinate blocks peripheral myelin formation
    Natalia Felitsyn
    Department of Neuroscience, College of Medicine, McKnight Brain Institute, University of Florida, Gainesville, Florida 32610 0244, USA
    J Neurochem 106:2068-79. 2008
    ..These results suggest that the neuropathic effects of delta-ALA are attributable, at least in part, to its pro-oxidant properties which damage myelinating Schwann cells...
  14. ncbi request reprint The methylenetetrahydrofolate reductase 677C->T polymorphism and dietary folate restriction affect plasma one-carbon metabolites and red blood cell folate concentrations and distribution in women
    Steven R Davis
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville, FL 32611 0370, USA
    J Nutr 135:1040-4. 2005
    ....
  15. pmc Human polymorphisms in the glutathione transferase zeta 1/maleylacetoacetate isomerase gene influence the toxicokinetics of dichloroacetate
    Albert L Shroads
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32610, USA
    J Clin Pharmacol 52:837-49. 2012
    ....
  16. pmc Role of dichloroacetate in the treatment of genetic mitochondrial diseases
    Peter W Stacpoole
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32610 0226, USA
    Adv Drug Deliv Rev 60:1478-87. 2008
    ..Recent in vitro data indicate that a combined DCA and gene therapy approach may also hold promise for the treatment of this devastating condition...
  17. pmc Production of 1-carbon units from glycine is extensive in healthy men and women
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville, FL 32611 0370, USA
    J Nutr 139:666-71. 2009
    ....
  18. ncbi request reprint Pharmacokinetics of oral dichloroacetate in dogs
    Herbert W Maisenbacher
    Small Animal Clinical Sciences, University of Florida, Gainesville, FL, 32610, USA
    J Biochem Mol Toxicol 27:522-5. 2013
    ..Dogs may be a useful model to further investigate the toxicokinetics and therapeutic potential of DCA. ..
  19. pmc Metabolomic analysis reveals extended metabolic consequences of marginal vitamin B-6 deficiency in healthy human subjects
    Jesse F Gregory
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville, Florida, United States of America
    PLoS ONE 8:e63544. 2013
    ..These findings demonstrate that marginal vitamin B-6 deficiency has widespread metabolic perturbations and illustrate the utility of metabolomics in evaluating complex effects of altered vitamin B-6 intake...
  20. ncbi request reprint Controlled clinical trial of dichloroacetate for treatment of congenital lactic acidosis in children
    Peter W Stacpoole
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida, Gainesville, Florida, USA
    Pediatrics 117:1519-31. 2006
    ..We tested this hypothesis by conducting the first double-blind, randomized, control trial of DCA in this disease...
  21. pmc Prenatal and postnatal expression of glutathione transferase ζ 1 in human liver and the roles of haplotype and subject age in determining activity with dichloroacetate
    Wenjun Li
    Department of Medicinal Chemistry, College of Pharmacy, University of Florida, Gainesville, FL 32610 0485, USA
    Drug Metab Dispos 40:232-9. 2012
    ..In summary, we report a neonatal onset and an age-related increase in GSTZ1 protein expression during human liver development. Haplotype influenced GSTZ1 activity with DCA but not protein expression...
  22. ncbi request reprint Inhibition and recovery of rat hepatic glutathione S-transferase zeta and alteration of tyrosine metabolism following dichloroacetate exposure and withdrawal
    Xu Guo
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida, FL 32610, USA
    Drug Metab Dispos 34:36-42. 2006
    ..The liver/body weight ratio increased in the high-dose group after 8 weeks of DCA. These studies demonstrate that short-term administration of DCA inhibits rat liver GSTz across the wide concentration range to which humans are exposed...
  23. ncbi request reprint The pyruvate dehydrogenase complex as a target for gene therapy
    Peter W Stacpoole
    Department of Medicine, Division of Endocrinology and Metabolism, University of Florida, College of Medicine, Gainesville, FL 32610, USA
    Curr Gene Ther 3:239-45. 2003
    ....
  24. ncbi request reprint Human kinetics of orally and intravenously administered low-dose 1,2-(13)C-dichloroacetate
    Minghong Jia
    General Clinical Research Center, University of Florida, 1600 Archer Road, Gainesville, Florida 32610, USA
    J Clin Pharmacol 46:1449-59. 2006
    ..Very little unchanged 1,2-(13)C-DCA was excreted in urine. Trace amounts of DCA alter its own kinetics after short-term exposure. These findings have important implications for interpreting the impact of this xenobiotic on human health...
  25. ncbi request reprint Plasma glutathione and cystathionine concentrations are elevated but cysteine flux is unchanged by dietary vitamin B-6 restriction in young men and women
    Steven R Davis
    Food Science andHuman Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville, FL 32611 0370, USA
    J Nutr 136:373-8. 2006
    ..These data indicate that glutathione homeostasis is altered by dietary vitamin B-6 deficiency and appears to be unrelated to cysteine flux under conditions of minimal amino acid intake as evaluated in this study...
  26. pmc Marginal vitamin B-6 deficiency decreases plasma (n-3) and (n-6) PUFA concentrations in healthy men and women
    Mei Zhao
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, niversity of Florida, Gainesville, FL, USA
    J Nutr 142:1791-7. 2012
    ..Such changes in blood lipids may be associated with the elevated risk of cardiovascular disease in vitamin B-6 insufficiency...
  27. pmc An animal model of PDH deficiency using AAV8-siRNA vector-mediated knockdown of pyruvate dehydrogenase E1α
    Carolyn Ojano-Dirain
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida, Gainesville, FL 32610, USA
    Mol Genet Metab 101:183-91. 2010
    ....
  28. pmc Mitochondrion as a novel site of dichloroacetate biotransformation by glutathione transferase zeta 1
    Wenjun Li
    Department of Medicinal Chemistry, College of Pharmacy, University of Florida, Gainesville, FL 32610 0485, USA
    J Pharmacol Exp Ther 336:87-94. 2011
    ..We conclude that the mitochondrion is a novel site of DCA biotransformation catalyzed by GSTZ1, an enzyme colocalized in cytosol and mitochondrial matrix...
  29. pmc Long-term safety of dichloroacetate in congenital lactic acidosis
    Monica Abdelmalak
    Department of Medicine, University of Florida College of Medicine, Gainesville, FL 32610, USA
    Mol Genet Metab 109:139-43. 2013
    ....
  30. ncbi request reprint Therapeutic potential of dichloroacetate for pyruvate dehydrogenase complex deficiency
    Kristen Berendzen
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida College of Medicine, P O Box 10226, Gainesville, FL 32610, USA
    Mitochondrion 6:126-35. 2006
    ..A controlled trial is needed to determine the definitive role of DCA in the management of this devastating disease...
  31. pmc The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients
    Kavi P Patel
    Department of Medicine Division of Endocrinology, Metabolism and Diabetes, College of Medicine, University of Florida, Gainesville, FL 32611, USA
    Mol Genet Metab 106:385-94. 2012
    ..There has been no recent comprehensive analysis of the natural history and clinical course of this disease...
  32. pmc Age-dependent kinetics and metabolism of dichloroacetate: possible relevance to toxicity
    Albert L Shroads
    Departments of Medicine, University of Florida Colleges of Medicine and Pharmacy, University of Florida, Gainesville, Florida, USA
    J Pharmacol Exp Ther 324:1163-71. 2008
    ..We conclude that age is an important variable in the in vivo metabolism and elimination of DCA and that it may account, in part, for the neurotoxicity of this compound in humans and other species...
  33. pmc Considerations for quantification of lipids in nerve tissue using matrix-assisted laser desorption/ionization mass spectrometric imaging
    Rachelle R Landgraf
    Department of Chemistry, University of Florida, Gainesville, FL 32611 7200, USA
    Rapid Commun Mass Spectrom 25:3178-84. 2011
    ..A precision of 5% or less standard error was achieved, illustrating that MALDI imaging offers a reliable means of in situ quantification for microgram-sized samples and requires minimal sample preparation...
  34. ncbi request reprint Unified gas chromatographic-mass spectrometric method for quantitating tyrosine metabolites in urine and plasma
    Albert L Shroads
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida, Gainesville, FL 32610, USA
    J Chromatogr B Analyt Technol Biomed Life Sci 808:153-61. 2004
    ..Thus far, the method has been successfully applied in the analysis of over 5000 urine and plasma samples from humans and rodents...
  35. ncbi request reprint Glycine turnover and decarboxylation rate quantified in healthy men and women using primed, constant infusions of [1,2-(13)C2]glycine and [(2)H3]leucine
    Yvonne Lamers
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville, FL 32611 0370, USA
    J Nutr 137:2647-52. 2007
    ..GCS is responsible for a substantial proportion of whole body glycine flux and constitutes a major route for the generation of 1-carbon units...
  36. ncbi request reprint Liquid chromatography-tandem mass spectrometry method for the simultaneous determination of delta-ALA, tyrosine and creatinine in biological fluids
    Natalia M Felitsyn
    Department of Medicine Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32610, USA
    Clin Chim Acta 350:219-30. 2004
    ..There is currently no facile, sensitive and specific method to measure these analytes simultaneously...
  37. ncbi request reprint Peripheral neuropathy in genetic mitochondrial diseases
    David E Stickler
    Department of Neurology, University of Florida, Gainesville, Florida, USA
    Pediatr Neurol 34:127-31. 2006
    ....
  38. ncbi request reprint Magnetic resonance spectroscopic investigation of mitochondrial fuel metabolism and energetics in cultured human fibroblasts: effects of pyruvate dehydrogenase complex deficiency and dichloroacetate
    Nicholas E Simpson
    Department of Medicine, Division of Endocrinology and Metabolism, University of Florida, Gainesville, FL 32610, USA
    Mol Genet Metab 89:97-105. 2006
    ..This study demonstrates that NMR spectroscopy provides insight into biochemical consequences of PDC deficiency and the mechanism of putative therapeutic agents...
  39. ncbi request reprint Pyruvate dehydrogenase complex deficiency caused by ubiquitination and proteasome-mediated degradation of the E1 subunit
    Zongchao Han
    Department of Pediatrics, Division of Cellular and Molecular Therapy, Molecular Genetics and Microbiology, University of Florida College of Medicine, Gainesville, FL 32610, USA
    J Biol Chem 283:237-43. 2008
    ..They also provide a novel mechanism accounting for congenital deficiency of the PDH complex and perhaps other inborn errors of metabolism...
  40. pmc Imaging of lipids in spinal cord using intermediate pressure matrix-assisted laser desorption-linear ion trap/Orbitrap MS
    Rachelle R Landgraf
    University of Florida, Gainesville, Florida 32611, USA
    Anal Chem 81:8488-95. 2009
    ..Employment of both mass analyzers provides a rapid and reliable means of compound identification based on MS/MS fragmentation and high-resolution mass spectrometry (HRMS) accurate mass...
  41. ncbi request reprint MALDI-linear ion trap microprobe MS/MS studies of the effects of dichloroacetate on lipid content of nerve tissue
    Rachelle R Landgraf
    Department of Chemistry, University of Florida, Gainesville, Florida 32611, USA
    Anal Chem 79:8170-5. 2007
    ..A marked decrease is observed in the intensity of lipid ions in spinal cord and sciatic nerve tissues from rats exposed to DCA. The results also demonstrate the rapid, sensitive, and semiquantitative capabilities of this method...
  42. pmc Down-regulation of expression of rat pyruvate dehydrogenase E1alpha gene by self-complementary adeno-associated virus-mediated small interfering RNA delivery
    Zongchao Han
    Department of Pediatrics, Division of Cellular and Molecular Therapy, University of Florida, College of Medicine, Gainesville, FL 32610, USA
    Mitochondrion 7:253-9. 2007
    ..Thus, scAAV siRNA-mediated knockdown of PDHA1 gene expression provides a strategy that may be applied to create a useful animal model of PDC deficiency...
  43. pmc Increased superoxide accumulation in pyruvate dehydrogenase complex deficient fibroblasts
    Lyudmyla G Glushakova
    Department of Medicine Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32611, USA
    Mol Genet Metab 104:255-60. 2011
    ....
  44. ncbi request reprint Differential metabolic effects of saturated versus polyunsaturated fats in ketogenic diets
    Brian S Fuehrlein
    Department of Medicine, College of Medicine, University of Florida, Gainesville, Florida 32610, USA
    J Clin Endocrinol Metab 89:1641-5. 2004
    ..Thus, a POLY KD may be superior to a classical SAT KD for chronic administration...
  45. ncbi request reprint Pharmacologic or genetic ablation of maleylacetoacetate isomerase increases levels of toxic tyrosine catabolites in rodents
    Chandramohan V Ammini
    Division of Endocrinology and Metabolism, Department of Medicine, University of Florida, Gainesville, FL 32610, USA
    Biochem Pharmacol 66:2029-38. 2003
    ..These results demonstrate that pharmacologic or genetic ablation of MAAI cause potentially toxic concentrations of tyrosine intermediates to accumulate in mice and perhaps in other species...
  46. ncbi request reprint Efficacy of dichloroacetate as a lactate-lowering drug
    Peter W Stacpoole
    Department of Medicine Division of Endocrinology and Metabolism, Biochemistry and Molecular Biology, College of Medicine, University of Florida, Gainesville, USA
    J Clin Pharmacol 43:683-91. 2003
    ..Recent clinical studies of acquired lactic acidosis suggest that DCA could be rapidly effective in reducing this risk factor of mortality in patients with mild hyperlactatemeia...
  47. ncbi request reprint Dietary vitamin B-6 restriction does not alter rates of homocysteine remethylation or synthesis in healthy young women and men
    Steven R Davis
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, University of Florida, Gainesville 32611 0370, USA
    Am J Clin Nutr 81:648-55. 2005
    ..The effects of vitamin B-6 status on steady-state kinetics of homocysteine metabolism in humans are unclear...
  48. pmc The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients
    Kavi P Patel
    Departments of Medicine Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Gainesville, FL 32611, USA
    Mol Genet Metab 105:34-43. 2012
    ..There has been no recent comprehensive analysis of the natural history and clinical course of this disease...
  49. pmc AAV3-mediated transfer and expression of the pyruvate dehydrogenase E1 alpha subunit gene causes metabolic remodeling and apoptosis of human liver cancer cells
    Lyudmyla G Glushakova
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, FL, USA
    Mol Genet Metab 98:289-99. 2009
    ..These data indicate that molecular targeting of mitochondrial oxidative metabolism in liver cancer cells by AAV3-mediated delivery of pda1 holds promise as a novel and effective therapeutic approach for human hepatic tumors...
  50. ncbi request reprint Exercise training, without weight loss, increases insulin sensitivity and postheparin plasma lipase activity in previously sedentary adults
    Glen E Duncan
    Department of Medicine, University of Florida, Gainesville, Florida 32610 0226, USA
    Diabetes Care 26:557-62. 2003
    ..To determine the effects of exercise, without weight loss, on insulin sensitivity (S(I)), postheparin plasma lipase activity (PHPL), intravenous fat clearance rate (K(2)), and fasting lipids in sedentary adults...
  51. ncbi request reprint Methylphenidate does not improve cognitive function in healthy sleep-deprived young adults
    Christopher L Bray
    University of Florida College of Medicine, Gainesville, FL 32610 1042, USA
    J Investig Med 52:192-201. 2004
    ..Abuse of methylphenidate, a treatment of attention-deficit/hyperactivity disorder, is reported to be increasing among students for the purpose of improving cognition...
  52. ncbi request reprint Gene therapy for pyruvate dehydrogenase E1alpha deficiency using recombinant adeno-associated virus 2 (rAAV2) vectors
    Renius Owen
    Department of Pediatrics, the General Clinical Research Center, University of Florida, Gainesville, Florida 32610, USA
    Mol Ther 6:394-9. 2002
    ..These studies provide the basis for future efforts to develop a recombinant AAV (rAAV)-based gene therapy approach for the correction of PDC deficiency...
  53. ncbi request reprint Dichloroacetate causes reversible demyelination in vitro: potential mechanism for its neuropathic effect
    Natalia Felitsyn
    Department of Neuroscience, College of Medicine, McKnight Brain Institute, University of Florida, Gainesville, Florida 32610 024, USA
    J Neurochem 100:429-36. 2007
    ..These results demonstrate that DCA induces a reversible inhibition of myelin-related proteins that may account, at least in part, for its clinical peripheral neuropathic effects...
  54. ncbi request reprint Methylenetetrahydrofolate reductase 677C->T polymorphism and folate status affect one-carbon incorporation into human DNA deoxynucleosides
    Eoin P Quinlivan
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Sciences, College of Medicine, University of Florida, Gainesville, FL 32611, USA
    J Nutr 135:389-96. 2005
    ..These findings support the feasibility of further assessment of factors affecting deoxynucleotide synthesis and DNA methylation in human 1-carbon metabolism...
  55. ncbi request reprint Renal manifestations of congenital lactic acidosis
    Richard E Neiberger
    Department of Pediatrics, Division of Nephrology, and the General Clinical Research Center, University of Florida, Gainesville, FL, USA
    Am J Kidney Dis 39:12-23. 2002
    ....
  56. ncbi request reprint Simultaneous determination of trace levels of nine haloacetic acids in biological samples as their pentafluorobenzyl derivatives by gas chromatography/tandem mass spectrometry in electron capture negative ion chemical ionization mode
    Minghong Jia
    Department of Medicine, Division of Endocrinology and Metabolism, College of Medicine, University of Florida, Box 100226, Gainesville, Florida 32610, USA
    Anal Chem 75:4065-80. 2003
    ..9-107.2%). The method was applied successfully to a kinetic investigation of low levels of HAAs in humans consuming chlorinated drinking water...
  57. ncbi request reprint Tracer-derived total and folate-dependent homocysteine remethylation and synthesis rates in humans indicate that serine is the main one-carbon donor
    Steven R Davis
    Food Science and Human Nutrition Department, Institute of Food and Agricultural Science, University of Florida, Gainesville, Florida 32611 0370, USA
    Am J Physiol Endocrinol Metab 286:E272-9. 2004
    ..Our dual-tracer procedure is well suited to measure the effects of nutrient deficiencies, genetic polymorphisms, and other metabolic perturbations on homocysteine synthesis and total and folate-dependent homocysteine remethylation...