Results 21 to 30 of about 47,907 (257)

Formation of reactive oxygen species by human and bacterial pyruvate and 2- oxoglutarate dehydrogenase multienzyme complexes reconstituted from recombinant components [PDF]

, 2015
Individual recombinant components of pyruvate and 2-oxoglutarate dehydrogenase multienzyme complexes (PDHc, OGDHc) of human and Escherichia coli (E. coli) origin were expressed and purified from E. coli with optimized protocols.
Adam-Vizi, Adam-Vizi, Adam-Vizi, Ambrus, Ambrus, Ambrus, Ambrus, Ambrus, Andreyev, Attila Ambrus, Azzi, Babady, Balakrishnan, Bando, Beata Torocsik, Berg, Blass, Brautigam, Brautigam, Brown, Bunik, Bunik, Cameron, Chinopoulos, Chinopoulos, Constantinescu, Contreras, Cruts, Diaz-Munoz, Droge, Dumont, Erfle, Fisher-Wellman, Frank, Frank Jordan, Gazaryan, Gibson, Gibson, Gibson, Gibson, Graf, Guest, Gupta, Hamada, Hansford, Harris, Hirashima, Hong, Huennekens, Kiselevsky, Klyachko, Koike, Koike, Korotchkina, Korotchkina, Kumar, Kussmaul, Lai, Lakaschus, Laszlo Tretter, Martinvalet, Massey, Massey, Massey, Mattias Nilsson, Mayo, McCord, McLennan, Mottley, Nakano, Natalia S. Nemeria, Nemeria, Nemeria, Nemeria, Nulton-Persson, Otulakowski, Patel, Perham, Pettit, Popov, Poulsen, Quinlan, Quinonez, Reed, Reed, Reed, Reed, Rosen, Saumweber, Seifert, Shaag, Shany, Sheu, Shi, Shim, Siebels, Song, Starkov, Starkov, Starkov, Tahara, Thekkumkara, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tyagi, Vaubel, Vera Adam-Vizi, Vereczki, Wei, Willems, Yan, Yang, Yi, Zundorf   +123 more
core   +1 more source

Genetic Inactivation of Pyruvate Dehydrogenase Kinases Improves Hepatic Insulin Resistance Induced Diabetes [PDF]

, 2013
Pyruvate dehydrogenase kinases (PDK1-4) play a critical role in the inhibition of the mitochondrial pyruvate dehydrogenase complex especially when blood glucose levels are low and pyruvate can be conserved for gluconeogenesis.
Dong, Xiaocheng C., Harris, Robert A., Tao, Rongya, White, Morris F., Xiong, Xiwen   +4 more
core   +7 more sources

A Case of Early Diagnosis of Pyruvate Dehydrogenase Complex Deficiency: The Use of Next-Generation Sequencing

Iranian Journal of Pediatrics, 2019
Introduction: Pyruvate dehydrogenase complex deficiency (PDHD) is a rare genetic mitochondrial disorder that is characterized by the broad clinical manifestations from lactic acidosis in neonate to chronic neurodegenerative conditions.
J. Kim, H. Kim, Ja-Hyun Jang, H. Jo, Kyu Hyung Lee   +4 more
semanticscholar   +1 more source

Neurophysiological profile of peripheral neuropathy associated with childhood mitochondrial disease [PDF]

, 2016
INTRODUCTION: Peripheral nerve involvement is common in mitochondrial disease but often unrecognised due to the prominent central nervous system features.
Bhattacharya, K, Christodoulou, J, Clark, D, Ellaway, C, Farrar, M, Menezes, MP, Ouvrier, R, Pitt, M, Rahman, S, Ryan, MM, Sampaio, H, Thorburn, DR, Ware, TL, Wedatilake, Y   +13 more
core   +1 more source

Programming Skeletal Muscle Metabolic Flexibility in Offspring of Male Rats in Response to Maternal Consumption of Slow Digesting Carbohydrates during Pregnancy [PDF]

, 2020
Skeletal muscle plays a relevant role in metabolic flexibility and fuel usage and the associated muscle metabolic inflexibility due to high-fat diets contributing to obesity and type 2 diabetes.
Andújar, Eloísa, Bueno Vargas, Pilar, Girón, María D., Manzano, Manuel, Martín, María J., Pérez Alegre, Mónica, Salto, Rafael, Vílchez, Jose D.   +7 more
core   +1 more source

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

Journal of Biological Chemistry, 2018
The pyruvate dehydrogenase multienzyme complex (PDHc) connects glycolysis to the tricarboxylic acid cycle by producing acetyl-CoA via the decarboxylation of pyruvate. Because of its pivotal role in glucose metabolism, this complex is closely regulated in
M. J. Whitley, P. Arjunan, N. Nemeria, L. Korotchkina, Yun-Hee Park, M. Patel, F. Jordan, W. Furey   +7 more
semanticscholar   +1 more source

Ambiguous Presentations of Pyruvate Dehydrogenase Deficiency: Combined Case Studies

, 2021
Background: The pyruvate dehydrogenase (PDH) complex is essential in the glycolytic conversion of pyruvate to acetyl-CoA. This reaction helps yield adenosine triphosphate (ATP) – a source for energy.
Mariam Hassan, A. Hassan, A. Shatila, Sudhir Kumar Chirakarra   +3 more
semanticscholar   +1 more source

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease [PDF]

, 2009
Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with lifethreatening cerebral oedema and dysmyelination in affected individuals.
Ara  jo, Ba  os, Cremer, Crome, Daniel, Dhopeshwarkar, Dodd, G. E. Homanics, H. S. Paul, Hariharan, Hauber, Homanics, J. Lazovic, J. M. Flanagan, Jan, K. C. Cheng, K. F. LaNoue, K. Griffin, K. J. Skvorak, Kamei, Killian, Le Masurier, Lowry, Lo  , M. C. Bewley, Mackenzie, McKean, Morton, O'Callaghan, Ouary, Patel, Patel, Regunathan, Schadewaldt, Schmithorst, Schonberger, Sgaravatti, She, SILBERMAN, Simon, Tews, W. J. Zinnanti, Wajner, Williamson, Yudkoff   +44 more
core   +2 more sources

Manifestations of X‐linked pyruvate dehydrogenase complex deficiency in female PDHA1 carriers

European Journal of Neurology
Pyruvate dehydrogenase complex deficiency is in up to 90% caused by pathogenic variants in the X‐linked PDHA1 gene. We aimed to investigate female relatives of index patients with PDHA1‐related disease to (i) describe the prevalence of female PDHA1 ...
A. Savvidou, K. Sofou, Erik A. Eklund, Johan Aronsson, Niklas Darin   +4 more
semanticscholar   +1 more source

Analysis of malaria associated genetic traits in Cabo Verde, a melting pot of European and sub Saharan settlers [PDF]

, 2009
Malaria has occurred in the Cabo Verde archipelago with epidemic characteristics since its colonization. Nowadays, it occurs in Santiago Island alone and though prophylaxis is not recommended by the World Health Organization, studies have highlight the ...
Alves, Joana, Amorim, António, Arez, Ana Paula, Faustino, Paula, Gonçalves, Nilza, Gusmão, Leonor, Machado, Patrícia, Manco, Licínio, Ribeiro, Letícia, Rosário, Virgílio Estólio do, Silva, João   +10 more
core   +1 more source