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Stimulating pyruvate dehydrogenase complex reduces itaconate levels and enhances TCA cycle anabolic bioenergetics in acutely inflamed monocytes.

Zhu X, Long D, Zabalawi M, Ingram B, Yoza BK, Stacpoole PW, McCall CE.

J Leukoc Biol. 2020 Jan 2. doi: 10.1002/JLB.3A1119-236R. [Epub ahead of print]


The changing landscape of clinical trials for mitochondrial diseases: 2011 to present.

Khayat D, Kurtz TL, Stacpoole PW.

Mitochondrion. 2020 Jan;50:51-57. doi: 10.1016/j.mito.2019.10.010. Epub 2019 Oct 25. Review.


Mitochondrial Glutathione Transferase Zeta 1 Is Inactivated More Rapidly by Dichloroacetate than the Cytosolic Enzyme in Adult and Juvenile Rat Liver.

Smeltz MG, Hu Z, Zhong G, Jahn SC, Rowland-Faux L, Horne LP, Stacpoole PW, James MO.

Chem Res Toxicol. 2019 Oct 21;32(10):2042-2052. doi: 10.1021/acs.chemrestox.9b00207. Epub 2019 Oct 1.


Dichloroacetate-induced peripheral neuropathy.

Stacpoole PW, Martyniuk CJ, James MO, Calcutt NA.

Int Rev Neurobiol. 2019;145:211-238. doi: 10.1016/bs.irn.2019.05.003. Epub 2019 Jun 11. Review.


Pyruvate dehydrogenase complex stimulation promotes immunometabolic homeostasis and sepsis survival.

McCall CE, Zabalawi M, Liu T, Martin A, Long DL, Buechler NL, Arts RJW, Netea M, Yoza BK, Stacpoole PW, Vachharajani V.

JCI Insight. 2018 Aug 9;3(15). pii: 99292. doi: 10.1172/jci.insight.99292. eCollection 2018 Aug 9.


Age-Related Changes in Expression and Activity of Human Hepatic Mitochondrial Glutathione Transferase Zeta1.

Zhong G, James MO, Smeltz MG, Jahn SC, Langaee T, Simpson P, Stacpoole PW.

Drug Metab Dispos. 2018 Aug;46(8):1118-1128. doi: 10.1124/dmd.118.081810. Epub 2018 May 31.


Personalized Dosing of Dichloroacetate Using GSTZ1 Clinical Genotyping Assay.

Langaee T, Wagner R, Horne LP, Lawson LA, Becker C, Shahin M, Starostik P, Stacpoole PW.

Genet Test Mol Biomarkers. 2018 Apr;22(4):266-269. doi: 10.1089/gtmb.2017.0261. Epub 2018 Mar 19.


Regulation of dichloroacetate biotransformation in rat liver and extrahepatic tissues by GSTZ1 expression and chloride concentration.

Jahn SC, Smeltz MG, Hu Z, Rowland-Faux L, Zhong G, Lorenzo RJ, Cisneros KV, Stacpoole PW, James MO.

Biochem Pharmacol. 2018 Jun;152:236-243. doi: 10.1016/j.bcp.2018.04.001. Epub 2018 Apr 5.


Response to Newman et al.

Parikh S, Goldstein A, Karaa A, Koenig MK, Anselm I, Brunel-Guitton C, Christodoulou J, Cohen BH, Dimmock D, Enns GM, Falk MJ, Feigenbaum A, Frye RE, Ganesh J, Griesemer D, Haas R, Horvath R, Korson M, Kruer MC, Mancuso M, McCormack S, Josee Raboisson M, Reimschisel T, Salvarinova R, Saneto RP, Scaglia F, Shoffner J, Stacpoole PW, Sue CM, Tarnopolsky M, Van Karnebeek C, Wolfe LA, Zolkipli Cunningham Z, Rahman S, Chinnery PF.

Genet Med. 2017 Dec;19(12). doi: 10.1038/gim.2017.164. Epub 2017 Oct 26. No abstract available.


Development of a novel observer reported outcome tool as the primary efficacy outcome measure for a rare disease randomized controlled trial.

Stacpoole PW, Shuster J, Thompson JLPS, Prather RA, Lawson LA, Zou B, Buchsbaum R, Nixon SJ.

Mitochondrion. 2018 Sep;42:59-63. doi: 10.1016/j.mito.2017.11.003. Epub 2017 Nov 10.


Therapeutic Targeting of the Pyruvate Dehydrogenase Complex/Pyruvate Dehydrogenase Kinase (PDC/PDK) Axis in Cancer.

Stacpoole PW.

J Natl Cancer Inst. 2017 Nov 1;109(11). doi: 10.1093/jnci/djx071. Review.


Model Informed Dose Optimization of Dichloroacetate for the Treatment of Congenital Lactic Acidosis in Children.

Mangal N, James MO, Stacpoole PW, Schmidt S.

J Clin Pharmacol. 2018 Feb;58(2):212-220. doi: 10.1002/jcph.1009. Epub 2017 Sep 15.


Patient care standards for primary mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society.

Parikh S, Goldstein A, Karaa A, Koenig MK, Anselm I, Brunel-Guitton C, Christodoulou J, Cohen BH, Dimmock D, Enns GM, Falk MJ, Feigenbaum A, Frye RE, Ganesh J, Griesemer D, Haas R, Horvath R, Korson M, Kruer MC, Mancuso M, McCormack S, Raboisson MJ, Reimschisel T, Salvarinova R, Saneto RP, Scaglia F, Shoffner J, Stacpoole PW, Sue CM, Tarnopolsky M, Van Karnebeek C, Wolfe LA, Cunningham ZZ, Rahman S, Chinnery PF.

Genet Med. 2017 Dec;19(12). doi: 10.1038/gim.2017.107. Epub 2017 Jul 27. Review.


Therapeutic applications of dichloroacetate and the role of glutathione transferase zeta-1.

James MO, Jahn SC, Zhong G, Smeltz MG, Hu Z, Stacpoole PW.

Pharmacol Ther. 2017 Feb;170:166-180. doi: 10.1016/j.pharmthera.2016.10.018. Epub 2016 Oct 19. Review.


Pharmacogenetic considerations with dichloroacetate dosing.

James MO, Stacpoole PW.

Pharmacogenomics. 2016 May;17(7):743-53. doi: 10.2217/pgs-2015-0012. Epub 2016 May 4. Review.


GSTZ1 expression and chloride concentrations modulate sensitivity of cancer cells to dichloroacetate.

Jahn SC, Solayman MH, Lorenzo RJ, Langaee T, Stacpoole PW, James MO.

Biochim Biophys Acta. 2016 Jun;1860(6):1202-10. doi: 10.1016/j.bbagen.2016.01.024. Epub 2016 Feb 2.


Vitamin B6 nutritional status and cellular availability of pyridoxal 5'-phosphate govern the function of the transsulfuration pathway's canonical reactions and hydrogen sulfide production via side reactions.

Gregory JF, DeRatt BN, Rios-Avila L, Ralat M, Stacpoole PW.

Biochimie. 2016 Jul;126:21-6. doi: 10.1016/j.biochi.2015.12.020. Epub 2016 Jan 4. Review.


Pyridoxine supplementation does not alter in vivo kinetics of one-carbon metabolism but modifies patterns of one-carbon and tryptophan metabolites in vitamin B-6-insufficient oral contraceptive users.

Rios-Avila L, Coats B, Ralat M, Chi YY, Midttun Ø, Ueland PM, Stacpoole PW, Gregory JF 3rd.

Am J Clin Nutr. 2015 Sep;102(3):616-25. doi: 10.3945/ajcn.115.113159. Epub 2015 Jul 22.


Chloride concentrations in human hepatic cytosol and mitochondria are a function of age.

Jahn SC, Rowland-Faux L, Stacpoole PW, James MO.

Biochem Biophys Res Commun. 2015 Apr 10;459(3):463-8. doi: 10.1016/j.bbrc.2015.02.128. Epub 2015 Mar 3.


The influence of human GSTZ1 gene haplotype variations on GSTZ1 expression.

Langaee TY, Zhong G, Li W, Hamadeh I, Solayman MH, McDonough CW, Stacpoole PW, James MO.

Pharmacogenet Genomics. 2015 May;25(5):239-45. doi: 10.1097/FPC.0000000000000129.


Sleep disorders associated with primary mitochondrial diseases.

Ramezani RJ, Stacpoole PW.

J Clin Sleep Med. 2014 Nov 15;10(11):1233-9. doi: 10.5664/jcsm.4212. Review.


Chloral hydrate, through biotransformation to dichloroacetate, inhibits maleylacetoacetate isomerase and tyrosine catabolism in humans.

Shroads AL, Coats BS, Langaee T, Shuster JJ, Stacpoole PW.

Drug Metab Pers Ther. 2015 Mar;30(1):49-55. doi: 10.1515/dmdi-2014-0015.


Dichloroacetate and cancer: new home for an orphan drug?

Kankotia S, Stacpoole PW.

Biochim Biophys Acta. 2014 Dec;1846(2):617-29. doi: 10.1016/j.bbcan.2014.08.005. Epub 2014 Aug 23. Review.


Haplotype variations in glutathione transferase zeta 1 influence the kinetics and dynamics of chronic dichloroacetate in children.

Shroads AL, Coats BS, McDonough CW, Langaee T, Stacpoole PW.

J Clin Pharmacol. 2015 Jan;55(1):50-5. doi: 10.1002/jcph.371. Epub 2014 Aug 6.


Chloride and other anions inhibit dichloroacetate-induced inactivation of human liver GSTZ1 in a haplotype-dependent manner.

Zhong G, Li W, Gu Y, Langaee T, Stacpoole PW, James MO.

Chem Biol Interact. 2014 May 25;215:33-9. doi: 10.1016/j.cbi.2014.02.015. Epub 2014 Mar 13.


Phase 1 trial of dichloroacetate (DCA) in adults with recurrent malignant brain tumors.

Dunbar EM, Coats BS, Shroads AL, Langaee T, Lew A, Forder JR, Shuster JJ, Wagner DA, Stacpoole PW.

Invest New Drugs. 2014 Jun;32(3):452-64. doi: 10.1007/s10637-013-0047-4. Epub 2013 Dec 3.


Pharmacokinetics of oral dichloroacetate in dogs.

Maisenbacher HW 3rd, Shroads AL 3rd, Zhong G, Daigle AD, Abdelmalak MM, Samper IS, Mincey BD, James MO, Stacpoole PW.

J Biochem Mol Toxicol. 2013 Dec;27(12):522-5. doi: 10.1002/jbt.21518. Epub 2013 Sep 13.


Metabolite profile analysis reveals functional effects of 28-day vitamin B-6 restriction on one-carbon metabolism and tryptophan catabolic pathways in healthy men and women.

da Silva VR, Rios-Avila L, Lamers Y, Ralat MA, Midttun Ø, Quinlivan EP, Garrett TJ, Coats B, Shankar MN, Percival SS, Chi YY, Muller KE, Ueland PM, Stacpoole PW, Gregory JF 3rd.

J Nutr. 2013 Nov;143(11):1719-27. doi: 10.3945/jn.113.180588. Epub 2013 Aug 21.


Expanding research to provide an evidence base for nutritional interventions for the management of inborn errors of metabolism.

Camp KM, Lloyd-Puryear MA, Yao L, Groft SC, Parisi MA, Mulberg A, Gopal-Srivastava R, Cederbaum S, Enns GM, Ershow AG, Frazier DM, Gohagan J, Harding C, Howell RR, Regan K, Stacpoole PW, Venditti C, Vockley J, Watson M, Coates PM.

Mol Genet Metab. 2013 Aug;109(4):319-28. doi: 10.1016/j.ymgme.2013.05.008. Epub 2013 May 23.


Metabolomic analysis reveals extended metabolic consequences of marginal vitamin B-6 deficiency in healthy human subjects.

Gregory JF 3rd, Park Y, Lamers Y, Bandyopadhyay N, Chi YY, Lee K, Kim S, da Silva V, Hove N, Ranka S, Kahveci T, Muller KE, Stevens RD, Newgard CB, Stacpoole PW, Jones DP.

PLoS One. 2013 Jun 11;8(6):e63544. doi: 10.1371/journal.pone.0063544. Print 2013.


Long-term safety of dichloroacetate in congenital lactic acidosis.

Abdelmalak M, Lew A, Ramezani R, Shroads AL, Coats BS, Langaee T, Shankar MN, Neiberger RE, Subramony SH, Stacpoole PW.

Mol Genet Metab. 2013 Jun;109(2):139-43. doi: 10.1016/j.ymgme.2013.03.019. Epub 2013 Apr 6.


Phenylbutyrate therapy for pyruvate dehydrogenase complex deficiency and lactic acidosis.

Ferriero R, Manco G, Lamantea E, Nusco E, Ferrante MI, Sordino P, Stacpoole PW, Lee B, Zeviani M, Brunetti-Pierri N.

Sci Transl Med. 2013 Mar 6;5(175):175ra31. doi: 10.1126/scitranslmed.3004986.


Design and implementation of the first randomized controlled trial of coenzyme CoQ₁₀ in children with primary mitochondrial diseases.

Stacpoole PW, deGrauw TJ, Feigenbaum AS, Hoppel C, Kerr DS, McCandless SE, Miles MV, Robinson BH, Tang PH.

Mitochondrion. 2012 Nov;12(6):623-9. doi: 10.1016/j.mito.2012.09.005. Epub 2012 Sep 25. Erratum in: Mitochondrion. 2013 Nov;13(6):953.


Marginal vitamin B-6 deficiency decreases plasma (n-3) and (n-6) PUFA concentrations in healthy men and women.

Zhao M, Lamers Y, Ralat MA, Coats BS, Chi YY, Muller KE, Bain JR, Shankar MN, Newgard CB, Stacpoole PW, Gregory JF 3rd.

J Nutr. 2012 Oct;142(10):1791-7. Epub 2012 Sep 5.


The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients.

Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW.

Mol Genet Metab. 2012 Jul;106(3):385-94.


The pyruvate dehydrogenase complex as a therapeutic target for age-related diseases.

Stacpoole PW.

Aging Cell. 2012 Jun;11(3):371-7. doi: 10.1111/j.1474-9726.2012.00805.x. Epub 2012 Apr 4. Review.


The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients.

Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW.

Mol Genet Metab. 2012 Jan;105(1):34-43. doi: 10.1016/j.ymgme.2011.09.032. Epub 2011 Oct 7. Review. Erratum in: Mol Genet Metab. 2012 Jul;106(3):384. Corrected and republished in: Mol Genet Metab. 2012 Jul;106(3):385-94.


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.

Li W, Gu Y, James MO, Hines RN, Simpson P, Langaee T, Stacpoole PW.

Drug Metab Dispos. 2012 Feb;40(2):232-9. doi: 10.1124/dmd.111.041533. Epub 2011 Oct 25.


Considerations for quantification of lipids in nerve tissue using matrix-assisted laser desorption/ionization mass spectrometric imaging.

Landgraf RR, Garrett TJ, Conaway MC, Calcutt NA, Stacpoole PW, Yost RA.

Rapid Commun Mass Spectrom. 2011 Oct 30;25(20):3178-84. doi: 10.1002/rcm.5189.


Increased superoxide accumulation in pyruvate dehydrogenase complex deficient fibroblasts.

Glushakova LG, Judge S, Cruz A, Pourang D, Mathews CE, Stacpoole PW.

Mol Genet Metab. 2011 Nov;104(3):255-60. doi: 10.1016/j.ymgme.2011.07.023. Epub 2011 Jul 28.


Development of optimized AAV3 serotype vectors: mechanism of high-efficiency transduction of human liver cancer cells.

Cheng B, Ling C, Dai Y, Lu Y, Glushakova LG, Gee SW, McGoogan KE, Aslanidi GV, Park M, Stacpoole PW, Siemann D, Liu C, Srivastava A, Ling C.

Gene Ther. 2012 Apr;19(4):375-84. doi: 10.1038/gt.2011.105. Epub 2011 Jul 21.


Human polymorphisms in the glutathione transferase zeta 1/maleylacetoacetate isomerase gene influence the toxicokinetics of dichloroacetate.

Shroads AL, Langaee T, Coats BS, Kurtz TL, Bullock JR, Weithorn D, Gong Y, Wagner DA, Ostrov DA, Johnson JA, Stacpoole PW.

J Clin Pharmacol. 2012 Jun;52(6):837-49. doi: 10.1177/0091270011405664. Epub 2011 Jun 3.


Why are there no proven therapies for genetic mitochondrial diseases?

Stacpoole PW.

Mitochondrion. 2011 Sep;11(5):679-85. doi: 10.1016/j.mito.2011.05.002. Epub 2011 May 13.


The dichloroacetate dilemma: environmental hazard versus therapeutic goldmine--both or neither?

Stacpoole PW.

Environ Health Perspect. 2011 Feb;119(2):155-8. doi: 10.1289/ehp.1002554. Epub 2010 Oct 4.


Mitochondrion as a novel site of dichloroacetate biotransformation by glutathione transferase zeta 1.

Li W, James MO, McKenzie SC, Calcutt NA, Liu C, Stacpoole PW.

J Pharmacol Exp Ther. 2011 Jan;336(1):87-94. doi: 10.1124/jpet.110.173195. Epub 2010 Sep 30.


An animal model of PDH deficiency using AAV8-siRNA vector-mediated knockdown of pyruvate dehydrogenase E1α.

Ojano-Dirain C, Glushakova LG, Zhong L, Zolotukhin S, Muzyczka N, Srivastava A, Stacpoole PW.

Mol Genet Metab. 2010 Oct-Nov;101(2-3):183-91. doi: 10.1016/j.ymgme.2010.07.008. Epub 2010 Jul 15.


Imaging of lipids in spinal cord using intermediate pressure matrix-assisted laser desorption-linear ion trap/Orbitrap MS.

Landgraf RR, Prieto Conaway MC, Garrett TJ, Stacpoole PW, Yost RA.

Anal Chem. 2009 Oct 15;81(20):8488-95. doi: 10.1021/ac901387u.


Peripheral neuropathy in rats exposed to dichloroacetate.

Calcutt NA, Lopez VL, Bautista AD, Mizisin LM, Torres BR, Shroads AL, Mizisin AP, Stacpoole PW.

J Neuropathol Exp Neurol. 2009 Sep;68(9):985-93. doi: 10.1097/NEN.0b013e3181b40217.

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