Results 21 to 30 of about 470,683 (177)

Genetic screening reveals phospholipid metabolism as a key regulator of the biosynthesis of the redox-active lipid coenzyme Q

Redox Biology, 2021
Mitochondrial energy production and function rely on optimal concentrations of the essential redox-active lipid, coenzyme Q (CoQ). CoQ deficiency results in mitochondrial dysfunction associated with increased mitochondrial oxidative stress and a range of
Anita Ayer, Daniel J. Fazakerley, Cacang Suarna, Ghassan J. Maghzal, Diba Sheipouri, Kevin J. Lee, Michelle C. Bradley, Lucía Fernández-del-Rio, Sergey Tumanov, Stephanie MY. Kong, Jelske N. van der Veen, Andrian Yang, Joshua W.K. Ho, Steven G. Clarke, David E. James, Ian W. Dawes, Dennis E. Vance, Catherine F. Clarke, René L. Jacobs, Roland Stocker   +19 more
doaj   +1 more source

CoQ Regulates Brown Adipose Tissue Respiration and Uncoupling Protein 1 Expression

Antioxidants, 2022
Coenzyme Q (CoQ, aka ubiquinone) is a key component of the mitochondrial electron transport chain (ETC) and membrane-incorporated antioxidant. CoQ10 deficiencies encompass a heterogeneous spectrum of clinical phenotypes and can be caused by hereditary ...
Ching-Fang Chang, Amanda L. Gunawan, Irene Liparulo, Peter-James H. Zushin, Ambre M. Bertholet, Yuriy Kirichok, Andreas Stahl   +6 more
doaj   +1 more source

Effects of inhibiting CoQ10 biosynthesis with 4-nitrobenzoate in human fibroblasts. [PDF]

PLoS ONE, 2012
Coenzyme Q(10) (CoQ(10)) is a potent lipophilic antioxidant in cell membranes and a carrier of electrons in the mitochondrial respiratory chain. We previously characterized the effects of varying severities of CoQ(10) deficiency on ROS production and ...
Catarina M Quinzii, Saba Tadesse, Ali Naini, Michio Hirano   +3 more
doaj   +1 more source

ADCK2 Haploinsufficiency Reduces Mitochondrial Lipid Oxidation and Causes Myopathy Associated with CoQ Deficiency. [PDF]

J Clin Med, 2019
Fatty acids and glucose are the main bioenergetic substrates in mammals. Impairment of mitochondrial fatty acid oxidation causes mitochondrial myopathy leading to decreased physical performance. Here, we report that haploinsufficiency of ADCK2, a member of the aarF domain-containing mitochondrial protein kinase family, in human is associated with liver
Vázquez-Fonseca L, Schaefer J, Navas-Enamorado I, Santos-Ocaña C, Hernández-Camacho JD, Guerra I, Cascajo MV, Sánchez-Cuesta A, Horvath Z, Siendones E, Jou C, Casado M, Gutiérrez P, Brea-Calvo G, López-Lluch G, Fernández-Ayala DJM, Cortés-Rodríguez AB, Rodríguez-Aguilera JC, Matté C, Ribes A, Prieto-Soler SY, Dominguez-Del-Toro E, Francesco AD, Aon MA, Bernier M, Salviati L, Artuch R, Cabo R, Jackson S, Navas P.   +29 more
europepmc   +7 more sources

Primary and secondary CoQ₁₀ deficiencies in humans [PDF]

BioFactors, 2011
AbstractCoQ10 deficiencies are clinically and genetically heterogeneous. This syndrome has been associated with five major clinical phenotypes: (1) encephalomyopathy, (2) severe infantile multisystemic disease, (3) cerebellar ataxia, (4) isolated myopathy, and (5) nephrotic syndrome. In a few patients, pathogenic mutations have been identified in genes
Catarina M, Quinzii, Michio, Hirano
openaire   +2 more sources

Predicting and Understanding the Pathology of Single Nucleotide Variants in Human COQ Genes

Antioxidants, 2022
Coenzyme Q (CoQ) is a vital lipid that functions as an electron carrier in the mitochondrial electron transport chain and as a membrane-soluble antioxidant.
Sining Wang, Akash Jain, Noelle Alexa Novales, Audrey N. Nashner, Fiona Tran, Catherine F. Clarke   +5 more
doaj   +1 more source

Minimal mitochondrial respiration is required to prevent cell death by inhibition of mTOR signaling in CoQ-deficient cells

Cell Death Discovery, 2021
Coenzyme Q (CoQ) is a lipid-like mobile electron transporter of the mitochondrial respiratory chain. Patients with partial loss-of-function mutations in the CoQ biosynthesis pathway suffer from partial primary CoQ deficiency (MIM 607426).
Ying Wang, Siegfried Hekimi
doaj   +1 more source

Neuroimaging in Primary Coenzyme-Q₁₀-Deficiency Disorders

Antioxidants, 2023
Coenzyme Q10 (CoQ10) is an endogenously synthesized lipid molecule. It is best known for its role as a cofactor within the mitochondrial respiratory chain where it functions in electron transfer and ATP synthesis.
Juliane Münch, Jannik Prasuhn, Lucia Laugwitz, Cheuk-Wing Fung, Brian H.-Y. Chung, Marcello Bellusci, Ertan Mayatepek, Dirk Klee, Felix Distelmaier   +8 more
doaj   +1 more source

ionathanch/coq: Is Sized Typing for Coq Practical? (JFP)

, 2021
This is the release for the supplementary materials for the submission of Is Sized Typing for Coq Practical? to the Journal of Functional Programming (JFP).
Jonathan Chan, The Coq Development Team
core   +1 more source

Mitochondrial electron transport chain, ceramide, and coenzyme Q are linked in a pathway that drives insulin resistance in skeletal muscle

eLife, 2023
Insulin resistance (IR) is a complex metabolic disorder that underlies several human diseases, including type 2 diabetes and cardiovascular disease. Despite extensive research, the precise mechanisms underlying IR development remain poorly understood ...
Alexis Diaz-Vegas, Søren Madsen, Kristen C Cooke, Luke Carroll, Jasmine XY Khor, Nigel Turner, Xin Y Lim, Miro A Astore, Jonathan C Morris, Anthony S Don, Amanda Garfield, Simona Zarini, Karin A Zemski Berry, Andrew P Ryan, Bryan C Bergman, Joseph T Brozinick, David E James, James G Burchfield   +17 more
doaj   +1 more source