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Select item 98960481.

Harvesting PdH Employing Pd Nano Icosahedrons via High Pressure

Kun Shi, Zihao Huo, Tianxiao Liang, Yongming Sui, Chuang Liu, Haiyun Shu, Lin Wang, Defang Duan, Bo Zou

Adv Sci (Weinh) 2023 Feb; 10(4): 2205133. Published online 2022 Nov 14. doi: 10.1002/advs.202205133

PMCID:: PMC9896048

Abstract Article PDF–1.4M

Select item 29373642.

The pdh Operon Is Expressed in a Subpopulation of Stationary-Phase Bacteria and Is Important for Survival of Sugar-Starved Streptococcus mutans

Monica Busuioc, Bettina A. Buttaro, Patrick J. Piggot

J Bacteriol. 2010 Sep; 192(17): 4395–4402. Published online 2010 Jun 25. doi: 10.1128/JB.00574-10

PMCID:: PMC2937364

Abstract Article PDF–2.2M

Select item 93208913.

Metal‐Doped PdH(111) Catalysts for CO₂ Reduction

Dr. Changzhi Ai, Prof. Tejs Vegge, Prof. Heine Anton Hansen

ChemSusChem. 2022 May 20; 15(10): e202200008. Published online 2022 Apr 8. doi: 10.1002/cssc.202200008

PMCID:: PMC9320891

Abstract Article PDF–2.5M

Select item 61099394.

Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the αV138M variant of human pyruvate dehydrogenase

Matthew J. Whitley, Palaniappa Arjunan, Natalia S. Nemeria, Lioubov G. Korotchkina, Yun-Hee Park, Mulchand S. Patel, Frank Jordan, William Furey

J Biol Chem. 2018 Aug 24; 293(34): 13204–13213. Published online 2018 Jul 3. doi: 10.1074/jbc.RA118.003996

PMCID:: PMC6109939

Abstract Article PDF–2.5M

Select item 48404315.

Difficulties in recognition of pyruvate dehydrogenase complex deficiency on the basis of clinical and biochemical features. The role of next-generation sequencing

E. Ciara, D. Rokicki, P. Halat, A. Karkucińska-Więckowska, D. Piekutowska-Abramczuk, J. Mayr, J. Trubicka, T. Szymańska-Dębińska, M. Pronicki, M. Pajdowska, M. Dudzińska, M. Giżewska, M. Krajewska-Walasek, J. Książyk, W. Sperl, R. Płoski, E. Pronicka

Mol Genet Metab Rep. 2016 Jun; 7: 70–76. Published online 2016 Apr 18. doi: 10.1016/j.ymgmr.2016.03.004

PMCID:: PMC4840431

Abstract Article PDF–634K

Select item 27979316.

Low Muscle Glycogen and Elevated Plasma Free Fatty Acid Modify but Do Not Prevent Exercise-Induced PDH Activation in Human Skeletal Muscle

Kristian Kiilerich, Mikkel Gudmundsson, Jesper B. Birk, Carsten Lundby, Sarah Taudorf, Peter Plomgaard, Bengt Saltin, Per A. Pedersen, Jorgen F.P. Wojtaszewski, Henriette Pilegaard

Diabetes. 2010 Jan; 59(1): 26–32. Published online 2009 Oct 15. doi: 10.2337/db09-1032

PMCID:: PMC2797931

Abstract Article PDF–166K

Select item 37548117.

The Spectrum of Pyruvate Dehydrogenase Complex Deficiency: Clinical, Biochemical and Genetic Features in 371 Patients

Kavi P. Patel, Thomas W. O'Brien, Sankarasubramon H. Subramony, Jonathan Shuster, Peter W. Stacpoole

Mol Genet Metab. Author manuscript; available in PMC 2013 Aug 27.

Published in final edited form as: Mol Genet Metab. 2012 Jan; 105(1): 34–43. Published online 2011 Oct 7. doi: 10.1016/j.ymgme.2011.09.032

Correction in:: volume 106 on page 384

PMCID:: PMC3754811

Abstract Article PDF–1.1M

Select item 49011788.

Beneficial effect of feeding a ketogenic diet to mothers on brain development in their progeny with a murine model of pyruvate dehydrogenase complex deficiency

Lioudmila Pliss, Urvi Jatania, Mulchand S. Patel

Mol Genet Metab Rep. 2016 Jun; 7: 78–86. Published online 2016 Apr 22. doi: 10.1016/j.ymgmr.2016.03.012

PMCID:: PMC4901178

Abstract Article PDF–1.2M

Select item 27219869.

A Korean Female Patient with Thiamine-responsive Pyruvate Dehydrogenase Complex Deficiency Due to a Novel Point Mutation (Y161C)in the PDHA1 Gene

Eun-Ha Lee, Mi-Sun Ahn, Jin-Soon Hwang, Kyung-Hwa Ryu, Sun-Jun Kim, Sung-Hwan Kim

J Korean Med Sci. 2006 Oct; 21(5): 800–804. Published online 2006 Oct 31. doi: 10.3346/jkms.2006.21.5.800

PMCID:: PMC2721986

Abstract Article PDF–107K

Select item 427086710.

Thiamine-Responsive and Non-responsive Patients with PDHC-E1 Deficiency: A Retrospective Assessment

Sanne van Dongen, Ruth M. Brown, Garry K. Brown, David R. Thorburn, Avihu Boneh

JIMD Rep. 2015; 15: 13–27. Published online 2014 Apr 10. doi: 10.1007/8904_2014_293

PMCID:: PMC4270867

Abstract Article PDF–216K

Select item 267031911.

The roles of aldehyde dehydrogenases (ALDHs) in the PDH bypass of Arabidopsis

Yanling Wei, Ming Lin, David J Oliver, Patrick S Schnable

BMC Biochem. 2009; 10: 7. Published online 2009 Mar 25. doi: 10.1186/1471-2091-10-7

PMCID:: PMC2670319

Abstract Article PDF–853K

Select item 757991412.

Pyruvate dehydrogenase complex deficiency: updating the clinical, metabolic and mutational landscapes in a cohort of Portuguese patients

Hana Pavlu-Pereira, Maria João Silva, Cristina Florindo, Sílvia Sequeira, Ana Cristina Ferreira, Sofia Duarte, Ana Luísa Rodrigues, Patrícia Janeiro, Anabela Oliveira, Daniel Gomes, Anabela Bandeira, Esmeralda Martins, Roseli Gomes, Sérgia Soares, Isabel Tavares de Almeida, João B. Vicente, Isabel Rivera

Orphanet J Rare Dis. 2020; 15: 298. Published online 2020 Oct 22. doi: 10.1186/s13023-020-01586-3

PMCID:: PMC7579914

Abstract Article PDF–1.5M

Select item 930254513.

Comparison Between Dichloroacetate and Phenylbutyrate Treatment for Pyruvate Dehydrogenase Deficiency

Patricia Karissa, Timothy Simpson, Simon P. Dawson, Teck Yew Low, Sook Hui Tay, Fatimah Diana Amin Nordin, Shamsul Mohd Zain, Pey Yee Lee, Yuh-Fen Pung

Br J Biomed Sci. 2022; 79: 10382. Published online 2022 May 19. doi: 10.3389/bjbs.2022.10382

PMCID:: PMC9302545

Abstract Article PDF–947K

Select item 258336814.

A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate

Zongchao Han, Kristen Berendzen, Li Zhong, Ira Surolia, Nitin Chouthai, Weihong Zhao, Njeri Maina, Arun Srivastava, Peter W Stacpoole

Mol Genet Metab. Author manuscript; available in PMC 2009 Apr 1.

Published in final edited form as: Mol Genet Metab. 2008 Apr; 93(4): 381–387. Published online 2008 Feb 21. doi: 10.1016/j.ymgme.2007.10.131

PMCID:: PMC2583368

Abstract Article PDF–1.4M

Select item 587421215.

Ketone Bodies as a Possible Adjuvant to Ketogenic Diet in PDHc Deficiency but Not in GLUT1 Deficiency

F. Habarou, N. Bahi-Buisson, E. Lebigot, C. Pontoizeau, M. T. Abi-Warde, A. Brassier, K. H. Le Quan Sang, C. Broissand, S. Vuillaumier-Barrot, A. Roubertie, A. Boutron, C. Ottolenghi, P. de Lonlay

JIMD Rep. 2018; 38: 53–59. Published online 2017 May 17. doi: 10.1007/8904_2017_30

PMCID:: PMC5874212

Abstract Article PDF–268K

Select item 254448716.

Tissue-specific pyruvate dehydrogenase complex deficiency causes cardiac hypertrophy and sudden death of weaned male mice

Sukhdeep Sidhu, Ashish Gangasani, Lioubov G. Korotchkina, Gen Suzuki, James A. Fallavollita, John M. Canty, Jr., Mulchand S. Patel

Am J Physiol Heart Circ Physiol. 2008 Sep; 295(3): H946–H952. Published online 2008 Jun 27. doi: 10.1152/ajpheart.00363.2008

PMCID:: PMC2544487

Abstract Article

Select item 1003878217.

Ketogenic diet in action: Metabolic profiling of pyruvate dehydrogenase deficiency

Eri Ogawa, Takako Hishiki, Noriyo Hayakawa, Hisato Suzuki, Kenjiro Kosaki, Makoto Suematsu, Toshiki Takenouchi

Mol Genet Metab Rep. 2023 Jun; 35: 100968. Published online 2023 Mar 20. doi: 10.1016/j.ymgmr.2023.100968

PMCID:: PMC10038782

Abstract Article PDF–1.6M

Select item 1007688718.

Enabling growth-decoupled Komagataella phaffii recombinant protein production based on the methanol-free P_DH promoter

Núria Bernat-Camps, Katharina Ebner, Veronika Schusterbauer, Jasmin Elgin Fischer, Miguel Angel Nieto-Taype, Francisco Valero, Anton Glieder, Xavier Garcia-Ortega

Front Bioeng Biotechnol. 2023; 11: 1130583. Published online 2023 Mar 23. doi: 10.3389/fbioe.2023.1130583

PMCID:: PMC10076887

Abstract Article PDF–1.8M

Select item 496714919.

Leslie A. Consitt, Gunjan Saxena, Alicson Saneda, Joseph A. Houmard

Am J Physiol Endocrinol Metab. 2016 Jul 1; 311(1): E145–E156. Published online 2016 May 24. doi: 10.1152/ajpendo.00452.2015

PMCID:: PMC4967149

Abstract Article

Select item 582862720.

Poldip2 is an oxygen-sensitive protein that controls PDH and αKGDH lipoylation and activation to support metabolic adaptation in hypoxia and cancer

Felipe Paredes, Kely Sheldon, Bernard Lassègue, Holly C. Williams, Elizabeth A. Faidley, Gloria A. Benavides, Gloria Torres, Fernanda Sanhueza-Olivares, Samantha M. Yeligar, Kathy K. Griendling, Victor Darley-Usmar, Alejandra San Martin

Proc Natl Acad Sci U S A. 2018 Feb 20; 115(8): 1789–1794. Published online 2018 Feb 6. doi: 10.1073/pnas.1720693115