Results 31 to 40 of about 42,696 (258)

BACE1 activity impairs neuronal glucose oxidation:rescue by beta-hydroxybutyrate and lipoic acid [PDF]

, 2015
Glucose hypometabolism and impaired mitochondrial function in neurons have been suggested to play early and perhaps causative roles in Alzheimer's disease (AD) pathogenesis. Activity of the aspartic acid protease, beta-site amyloid precursor protein (APP)
Arsenian, Bateman, Beal, Bielarczyk, Blass, Bogaert, Bosetti, Bouzier-Sore, Bubber, Butterworth, Casley, Casley, Caspersen, Coleman, Costantini, Danial, David L. Hamilton, DeKosky, DeKosky, Devi, Du, Fang, Forero, Fukumoto, Geula, Gibson, Giménez-Cassina, Guglielmotto, Gómez-Isla, Hager, Hamilton, Harada, Henderson, Henderson, Hirai, Holsinger, Huang, Hussain, Itoh, Jack, Jack, Jack, Jha, John A. Findlay, Johnson, Kankotia, Kawaja, Kim, Knopman, Ko, Korolainen, Lei, Li, Lustbader, Magistretti, Manczak, Martiskainen, Masdeu, Mastrogiacoma, Maurer, McKenna, Meakin, Michael L. J. Ashford, Mirshamsi, Mosconi, Mosconi, Mowrer, Mucke, Mullen, Nelson, Nilsen, Nunomura, O'Connor, Packer, Parker, Patel, Pereira, Perry, Phypers, Puglielli, Reger, Reiman, Sancheti, Shankar, Shearman, Sinha, Small, Sokoloff, Sorbi, Sowell, Swerdlow, Tyler, Vaishnavi, Vassar, Vassar, Velliquette, Vlassenko, von Kienlin, Wilkins, Wischmeyer, Wise, Xun, Yang, Yao, Yao, Zaidan, Zhang, Zhang, Zhao   +108 more
core   +5 more sources

Red Blood Cell Membrane Conductance in Hereditary Haemolytic Anaemias

Frontiers in Physiology, 2019
Congenital haemolytic anaemias are inherited disorders caused by red blood cell membrane and cytoskeletal protein defects, deviant hemoglobin synthesis and metabolic enzyme deficiencies.
Polina Petkova-Kirova, Laura Hertz, Laura Hertz, Jens Danielczok, Rick Huisjes, Asya Makhro, Anna Bogdanova, Maria del Mar Mañú-Pereira, Joan-Lluis Vives Corrons, Richard van Wijk, Lars Kaestner, Lars Kaestner   +11 more
doaj   +1 more source

Clinical, biochemical, and genetic spectrum of seven patients with NFU1 deficiency [PDF]

, 2015
Disorders of the mitochondrial energy metabolism are clinically and genetically heterogeneous. An increasingly recognized subgroup is caused by defective mitochondrial iron-sulfur (Fe-S) cluster biosynthesis, with defects in 13 genes being linked to ...
Angela eHagendorff, Arnaud V Vanlander, Barbara ePlecko, Boris eRolinski, Charlotte L Alston, Christine eMakowski, Franz A Zimmermann, Holger eProkisch, Holger eProkisch, Johannes A Mayr, Joél eSmet, Lucia eAbela, Maja eHempel, Marianne eRohrbach, Peter eFreisinger, Robert W Taylor, Rudy eVan Coster, Saikat eSantra, Sara eSeneka, Stephanie eSpranger, Steven A Hardy, Thomas eMeitinger, Thomas eMeitinger, Tobias Bernd Haack, Tobias Bernd Haack, Uwe eAhting, Wolfgang eSperl   +26 more
core   +3 more sources

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

Erythrocytes as Carriers of Therapeutic Enzymes. [PDF]

, 2020
Therapeutic enzymes are administered for the treatment of a wide variety of diseases. They exert their effects through binding with a high affinity and specificity to disease-causing substrates to catalyze their conversion to a non-noxious product, to ...
Bax, BE
core   +1 more source

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

Proteogenomic characterization of cholangiocarcinoma

Hepatology, EarlyView., 2022
Proteogenomic characterization of cholangiocarcinoma with therapeutic strategies Abstract Background and Aims Cholangiocarcinoma (CCA) is a highly heterogeneous cancer with limited understanding and few effective therapeutic approaches. We aimed at providing a proteogenomic CCA characterization to inform biological processes and treatment ...
Mengjie Deng, Peng Ran, Lingli Chen, Yunzhi Wang, Zixiang Yu, Ke Cai, Jinwen Feng, Zhaoyu Qin, Yanan Yin, Subei Tan, Yang Liu, Chen Xu, Guoming Shi, Yuan Ji, Jian‐Yuan Zhao, Jian Zhou, Jia Fan, Yingyong Hou, Chen Ding   +18 more
wiley   +1 more source

Role of Esrrg in the Fibrate-Mediated Regulation of Lipid Metabolism Genes in Human ApoA-I Transgenic Mice [PDF]

, 2009
We have used a new ApoA-I transgenic mouse model to identify by global gene expression profiling, candidate genes that affect lipid and lipoprotein metabolism in response to fenofibrate treatment.
A Chroni, A Duka, A Patsialou, A Rigotti, A Walsh, AP Feinberg, B Staels, B Staels, B Staels, B Staels, B Staels, B Staels, BN Finck, CW Miller, D Arsenijevic, D Lan, D Sanoudou, D Sanoudou, D Sanoudou, EM Rubin, ES Cathcart, F Yadetie, G Fredrikson, G Martin, H Duez, H Samyn, HB Rubins, HY Kan, J Karbowska, J Lin, J St Pierre, JC Fruchart, JD Brunzell, JJ Lehman, JK Reddy, JL Goldstein, JM Huss, JM Huss, JP Desager, K C Hayes, K Drosatos, K Motojima, K Schoonjans, K Schoonjans, KE Kypreos, KE Kypreos, KS Frederiksen, L Berthou, LA Pennacchio, LJ Kelly, M Bouly, MB Eisen, MC Hunt, MJ Holness, N Hennuyer, N Vu-Dac, N Vu-Dac, N Vu-Dac, N Vu-Dac, NF Cariello, P Puigserver, Q Guo, R Sladek, RB Vega, RL Jacobs, SA Greenberg, SN Schreiber, V Beisvag, V Giguere, V I Zannis, V Litvak, VG Tusher, VI Zannis, VI Zannis, VI Zannis, WC Roberts, X Prieur, Y Kamei, Y Nagai, Z Wu   +79 more
core   +3 more sources

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

Essential lipid autacoids rewire mitochondrial energy efficiency in metabolic dysfunction‐associated fatty liver disease

Hepatology, EarlyView., 2022
Increased liver content of DHA‐derived small lipid autacoids (i.e resolvin D1 and maresin 1) associates with enhanced mitochondrial oxidative phosphorylation, fatty acid β‐oxidation and bioenergetic metabolic flux. These features provide hepatic protection from steatotic, pro‐inflammatory and fibrogenic insults.
Cristina López‐Vicario, David Sebastián, Mireia Casulleras, Marta Duran‐Güell, Roger Flores‐Costa, Ferran Aguilar, Juan José Lozano, Ingrid W. Zhang, Esther Titos, Jing X. Kang, Antonio Zorzano, Makoto Arita, Joan Clària   +12 more
wiley   +1 more source