Coenzyme Q and mitochondrial disease
CM Quinzii, M Hirano - Developmental disabilities research …, 2010 - Wiley Online Library
Abstract Coenzyme Q10 (CoQ10) is an essential electron carrier in the mitochondrial
respiratory chain and an important antioxidant. Deficiency of CoQ10 is a clinically and …
respiratory chain and an important antioxidant. Deficiency of CoQ10 is a clinically and …
[PDF][PDF] COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency
Primary coenzyme Q10 (CoQ 10) deficiencies are rare, clinically heterogeneous disorders
caused by mutations in several genes encoding proteins involved in CoQ 10 biosynthesis …
caused by mutations in several genes encoding proteins involved in CoQ 10 biosynthesis …
Mutations in COQ4, an essential component of coenzyme Q biosynthesis, cause lethal neonatal mitochondrial encephalomyopathy
Background The identification of the molecular basis of mitochondrial disorders continues to
be challenging and expensive. The increasing usage of next-generation sequencing is …
be challenging and expensive. The increasing usage of next-generation sequencing is …
CoQ10 deficiency diseases in adults
CM Quinzii, M Hirano, S DiMauro - Mitochondrion, 2007 - Elsevier
Deficiency of Coenzyme Q10 (CoQ10) in muscle has been associated with a spectrum of
diseases including infantile-onset multi-systemic diseases, encephalomyopathies with …
diseases including infantile-onset multi-systemic diseases, encephalomyopathies with …
[HTML][HTML] Coenzyme Q biosynthesis in health and disease
MJ Acosta, LV Fonseca, MA Desbats, C Cerqua… - … et Biophysica Acta (BBA …, 2016 - Elsevier
Coenzyme Q (CoQ, or ubiquinone) is a remarkable lipid that plays an essential role in
mitochondria as an electron shuttle between complexes I and II of the respiratory chain, and …
mitochondria as an electron shuttle between complexes I and II of the respiratory chain, and …
Secondary CoQ10 deficiency, bioenergetics unbalance in disease and aging
P Navas, MV Cascajo, M Alcázar‐Fabra… - Biofactors, 2021 - Wiley Online Library
Abstract Coenzyme Q10 (CoQ10) deficiency is a rare disease characterized by a decreased
accumulation of CoQ10 in cell membranes. Considering that CoQ10 synthesis and most of …
accumulation of CoQ10 in cell membranes. Considering that CoQ10 synthesis and most of …
A fatal case of COQ7‐associated primary coenzyme Q10 deficiency
AKY Kwong, ATG Chiu, MHY Tsang, KS Lun… - JIMD …, 2019 - Wiley Online Library
Abstract Background Primary coenzyme Q10 (CoQ10) deficiencies are clinically and
genetically heterogeneous group of disorders associated with defects of genes involved in …
genetically heterogeneous group of disorders associated with defects of genes involved in …
Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency
L Garcia-Corzo, M Luna-Sánchez… - Human molecular …, 2013 - academic.oup.com
Abstract Coenzyme Q10 (CoQ10) or ubiquinone is a well-known component of the
mitochondrial respiratory chain. In humans, CoQ10 deficiency causes a mitochondrial …
mitochondrial respiratory chain. In humans, CoQ10 deficiency causes a mitochondrial …
Coenzyme Q10 Therapy
J Garrido-Maraver, MD Cordero… - Molecular …, 2014 - karger.com
For a number of years, coenzyme Q10 (CoQ10) was known for its key role in mitochondrial
bioenergetics; later studies demonstrated its presence in other subcellular fractions and in …
bioenergetics; later studies demonstrated its presence in other subcellular fractions and in …
[PDF][PDF] A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial …
AJ Duncan, M Bitner-Glindzicz, B Meunier… - The American Journal of …, 2009 - cell.com
Coenzyme Q 10 is a mobile lipophilic electron carrier located in the inner mitochondrial
membrane. Defects of coenzyme Q 10 biosynthesis represent one of the few treatable …
membrane. Defects of coenzyme Q 10 biosynthesis represent one of the few treatable …