Results 1 to 10 of about 470,683 (177)

The Q-Junction and the Inflammatory Response Are Critical Pathological and Therapeutic Factors in CoQ Deficiency [PDF]

Redox Biology, 2022
Defects in Coenzyme Q (CoQ) metabolism have been associated with primary mitochondrial disorders, neurodegenerative diseases and metabolic conditions.
Agustin Hidalgo-Gutierrez, Eliana Barriocanal-Casado, Albert J.R. Heck   +2 more
exaly   +15 more sources

The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency [PDF]

Frontiers in Physiology, 2017
Coenzyme Q (CoQ) is a lipid present in all cell membranes. One of the multiple metabolic functions of CoQ is to transport electrons in the reaction catalyzed by sulfide:quinone oxidoreductase (SQOR), the first enzyme of the oxidation pathway of sulfides (
Agustin Hidalgo-Gutierrez, Giulio Kleiner, Luis C. López   +2 more
exaly   +10 more sources

CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome [PDF]

EMBO Molecular Medicine, 2016
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several other functions in the cellular metabolism. One of them is to function as an electron carrier in the reaction catalyzed by sulfide:quinone oxidoreductase (
Marta Luna‐Sánchez, Agustín Hidalgo‐Gutiérrez, Tatjana M Hildebrandt, Julio Chaves‐Serrano, Eliana Barriocanal‐Casado, Ángela Santos‐Fandila, Miguel Romero, Ramy KA Sayed, Juan Duarte, Holger Prokisch, Markus Schuelke, Felix Distelmaier, Germaine Escames, Darío Acuña‐Castroviejo, Luis C López   +14 more
doaj   +10 more sources

Mitochondrial CoQ deficiency is a common driver of mitochondrial oxidants and insulin resistance [PDF]

eLife, 2018
Insulin resistance in muscle, adipocytes and liver is a gateway to a number of metabolic diseases. Here, we show a selective deficiency in mitochondrial coenzyme Q (CoQ) in insulin-resistant adipose and muscle tissue.
Daniel J Fazakerley, Rima Chaudhuri, Pengyi Yang, Ghassan J Maghzal, Kristen C Thomas, James R Krycer, Sean J Humphrey, Benjamin L Parker, Kelsey H Fisher-Wellman, Christopher C Meoli, Nolan J Hoffman, Ciana Diskin, James G Burchfield, Mark J Cowley, Warren Kaplan, Zora Modrusan, Ganesh Kolumam, Jean YH Yang, Daniel L Chen, Dorit Samocha-Bonet, Jerry R Greenfield, Kyle L Hoehn, Roland Stocker, David E James   +23 more
doaj   +8 more sources

Brown Adipose Tissue CoQ Deficiency Activates the Integrated Stress Response and FGF21-Dependent Mitohormesis [PDF]

EMBO Journal
Coenzyme Q (CoQ) is essential for mitochondrial respiration and required for thermogenic activity in brown adipose tissues (BAT). CoQ deficiency leads to a wide range of pathological manifestations, but mechanistic consequences of CoQ deficiency in ...
Gunes Parlakgul, Irene Liparulo, Andreas Stahl   +2 more
exaly   +5 more sources

Manganese-driven CoQ deficiency [PDF]

Nature Communications, 2022
Across phylae, excess manganese disrupts energy metabolism by unclear mechanisms. Here, Diessl et al. report that failure of mitochondrial bioenergetics upon manganese overload is due to mismetallation of a diiron enzyme crucial for CoQ ...
Jutta Diessl, Jens Berndtsson, Filomena Broeskamp, Lukas Habernig, Verena Kohler, Carmela Vazquez-Calvo, Arpita Nandy, Carlotta Peselj, Sofia Drobysheva, Ludovic Pelosi, F.-Nora Vögtle, Fabien Pierrel, Martin Ott, Sabrina Büttner   +13 more
doaj   +8 more sources

The treatment of primary CoQ deficiency requires the targeting of multiple pathogenic mechanisms [PDF]

Communications Medicine
Background Primary coenzyme Q (CoQ) deficiency is a severe mitochondrial disorder characterized by diverse clinical manifestations due to multiple pathomechanisms. Although CoQ10 supplementation remains the standard treatment, its therapeutic efficacy is
Pilar González-García, Laura Jiménez-Sánchez, Julia Corral-Sarasa, Sergio López-Herrador, Sara Torres-Rusillo, María Elena Díaz-Casado, Luis C. López   +6 more
doaj   +6 more sources

Cellular Models for Primary CoQ Deficiency Pathogenesis Study [PDF]

International Journal of Molecular Sciences, 2021
Primary coenzyme Q10 (CoQ) deficiency includes a heterogeneous group of mitochondrial diseases characterized by low mitochondrial levels of CoQ due to decreased endogenous biosynthesis rate. These diseases respond to CoQ treatment mainly at the early stages of the disease.
Guillermo López-Lluch, Maria Victoria Cascajo-Almenara, Carlos Santos   +2 more
exaly   +9 more sources

Spatial mapping of CoQ10 repletion by BPM31510 in a genetic mouse model (Coq4F147C) of coenzyme Q deficiency [PDF]

Journal of Lipid Research
Primary coenzyme Q10 (CoQ10) deficiency is a rare mitochondrial disorder caused by mutations in genes involved in CoQ biosynthesis (e.g., COQ4) that result in impaired mitochondrial respiration, oxidative stress, and dysfunction across multiple organ ...
Eliana Barriocanal-Casado, Sylwia A. Stopka, Alba Pesini, Juan J. Aristizabal-Henao, Srada Karmacharya, Devon Van Cura, Ryan Zhang, Kelsey R. Nickerson, Kashni Grover, Oksana Zavidij, Sarah R. Wessel, Niven R. Narain, Vijay Modur, Stephane Gesta, Michael A. Kiebish, Catarina M. Quinzii   +15 more
doaj   +2 more sources

Biosynthesis, Deficiency, and Supplementation of Coenzyme Q

Antioxidants, 2023
Originally identified as a key component of the mitochondrial respiratory chain, Coenzyme Q (CoQ or CoQ10 for human tissues) has recently been revealed to be essential for many different redox processes, not only in the mitochondria, but elsewhere within
Laura García-Corzo, Carmine Staiano, Nadia Turton   +2 more
exaly   +3 more sources