Treatment of CoQ10 Deficient Fibroblasts with Ubiquinone, CoQ Analogs, and Vitamin C: Time- and Compound-Dependent Effects

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

(2010) Catarina M. Quinzii et al.   FASEB JOURNAL

A Nonsense Mutation in COQ9 Causes Autosomal-Recessive Neonatal-Onset Primary Coenzyme Q10 Deficiency: A Potentially Treatable Form of Mitochondrial Disease

(2009) Andrew J. Duncan et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III

(2009) Petra Kaufmann et al.   ANNALS OF NEUROLOGY

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy

(2009) Ángeles Rodríguez-Hernández et al.   Autophagy

Human CoQ10deficiencies

(2009) C. M. Quinzii et al.   BIOFACTORS

Coenzyme Q10 in Neuromuscular and Neurodegenerative Disorders

(2009) M. Mancuso et al.   CURRENT DRUG TARGETS

Clinical experience with high-dose idebenone in Friedreich ataxia

(2009) Jörg B. Schulz et al.   JOURNAL OF NEUROLOGY

ADCK3, an Ancestral Kinase, Is Mutated in a Form of Recessive Ataxia Associated with Coenzyme Q10 Deficiency

(2008) Clotilde Lagier-Tourenne et al.   AMERICAN JOURNAL OF HUMAN GENETICS

CABC1 Gene Mutations Cause Ubiquinone Deficiency with Cerebellar Ataxia and Seizures

(2008) Julie Mollet et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Ubiquinone and tocopherol: Dissimilar siblings

(2008) L. Gille et al.   BIOCHEMICAL PHARMACOLOGY

Differential effects of mitochondrial Complex I inhibitors on production of reactive oxygen species

(2008) Romana Fato et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

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

(2008) Catarina M. Quinzii et al.   FASEB JOURNAL

Clk-1 deficiency induces apoptosis associated with mitochondrial dysfunction in mouse embryos

(2008) Mayumi Takahashi et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Early Coenzyme Q10 Supplementation in Primary Coenzyme Q10 Deficiency

(2008) Giovanni Montini et al.   NEW ENGLAND JOURNAL OF MEDICINE

Detection of 2-hydroxyethidium in cellular systems: a unique marker product of superoxide and hydroethidine

(2007) Jacek Zielonka et al.   Nature Protocols