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Coenzyme Q and mitochondrial disease

published in: Developmental Disabilities Research Reviews

date of publication: 2010-01-01

language: English

main subject: mitochondrion, mitochondrial disease

Cites articles 73

Date

Title

Authors

2010-07-30

Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects

Shamima Rahman
2010-05-21

Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency

Clotilde Lagier-Tourenne, Salvatore DiMauro, Filippo Maria Santorelli, Shamima Rahman
2009-11-27

Coenzyme Q10 is frequently reduced in muscle of patients with mitochondrial myopathy

Ségolène Aymé
2009-06-22

Biosynthesis and bioproduction of coenzyme Q10 by yeasts and other organisms
2009-06-01

Short-patch single-strand break repair in ataxia oculomotor apraxia-1.
2009-05

A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease

Michael I Sadowski, Brigitte Meunier, Luis C. López, John Anthony Hardy, Andrew Singleton, Shamima Rahman
2009-03

ETFDH mutations, CoQ10 levels, and respiratory chain activities in patients with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency

Luis C. López, Ichizo Nishino
2009-01-01

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy
2009-03-01

Reduction of hydrophilic ubiquinones by the flavin in mitochondrial NADH:ubiquinone oxidoreductase (Complex I) and production of reactive oxygen species

Martin S King, Judy Hirst
2009-03-01

Clinical and genetic analysis of lipid storage myopathies

Luis C. López, Ichizo Nishino
2008-12

Ubiquinone biosynthesis in Saccharomyces cerevisiae: the molecular organization of O-methylase Coq3p depends on Abc1p/Coq8p
2008-09-10

Coenzyme Q10 supplementation rescues renal disease in Pdss2kd/kd mice with mutations in prenyl diphosphate synthase subunit 2

Catherine F. Clarke
2008-04-25

Primary coenzyme Q deficiency in Pdss2 mutant mice causes isolated renal disease

Volker H. Haase, Marni Falk, Catherine F. Clarke
2008-03

ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency

Jean-Louis Mandel, Clotilde Lagier-Tourenne, Mario H. Barros, Christelle Thibault, Mathieu Anheim, Luis C. López, Christine Tranchant, Salvatore DiMauro, Frédéric Plewniak
2008-03

CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures

Nathalie Boddaert, Dominique Chrétien, Agnès Rötig, Arnold Munnich, Hélène Ogier de Baulny, Isabelle Desguerre, Agnès Delahodde, Julie Mollet, Valérie Serre, Pascale de Lonlay
2008-01-29

Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency

Salvatore DiMauro
2008-01-01

Human CoQ10 deficiencies
2008-02-02

Systematic evaluation of muscle coenzyme Q10 content in children with mitochondrial respiratory chain enzyme deficiencies

Kevin E. Bove
2008-06-01

Early coenzyme Q10 supplementation in primary coenzyme Q10 deficiency

Giovanni Montini
2007-09-12

COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement

Enrico Bertini, Gianluca Caridi, Anna Pastore, Fiorella Piemonte, Luisa Murer, Giovanni Montini, Andrea Onetti Muda, Gian Marco Ghiggeri, Filippo Maria Santorelli, Francesca Diomedi Camassei, Laura Barisoni
2007-03-16

The antioxidant role of coenzyme Q.

Kerstin Brismar
2007-03-30

Endogenous synthesis of coenzyme Q in eukaryotes
2007-04-05

Enrichment of coenzyme Q10 in plasma and blood cells: defense against oxidative damage
2007-03-16

The uptake and distribution of coenzyme Q10.
2007-08

The myopathic form of coenzyme Q10 deficiency is caused by mutations in the electron-transferring-flavoprotein dehydrogenase (ETFDH) gene

Holger Prokisch, Rita Horvath, Hanns Lochmüller, Haluk Topaloglu, Salvatore DiMauro, Benedikt Schoser
2007-03-20

Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis

Giovanni Montini, Angeles Rodriguez-Hernandez, Mario D Cordero, José A Sánchez-Alcázar, C Santos-Ocaña
2007-03

Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders

Dominique Chrétien, Agnès Rötig, Arnold Munnich
2007-08-20

Cardiofaciocutaneous (CFC) syndrome associated with muscular coenzyme Q10 deficiency
2007-01-01

Muscle coenzyme Q10 deficiencies in ataxia with oculomotor apraxia 1
2006-10-27

Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations

Luis C. López, Tomotake Kanki, Richard J Rodenburg
2006-05-01

Coenzyme Q10: absorption, tissue uptake, metabolism and pharmacokinetics
2006-02

A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency

Article - wd:Q37785517

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