Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice

Allogeneic haematopoietic stem cell transplantation for mitochondrial neurogastrointestinal encephalomyopathy

(2015) Joerg P. Halter et al.   BRAIN

Gene therapy returns to centre stage

(2015) Luigi Naldini   NATURE

Differentiation of Human Neural Stem Cells into Motor Neurons Stimulates Mitochondrial Biogenesis and Decreases Glycolytic Flux

(2015) Laura C. O'Brien et al.   STEM CELLS AND DEVELOPMENT

Gene therapy and peripheral nerve repair: a perspective

(2015) Stefan A. Hoyng et al.   Frontiers in Molecular Neuroscience

Correction of Methylmalonic Aciduria In Vivo Using a Codon-Optimized Lentiviral Vector

(2014) Edward S.Y. Wong et al.   HUMAN GENE THERAPY

Regulation of coenzyme Q biosynthesis in yeast: A new complex in the block

(2014) Isabel González-Mariscal et al.   IUBMB LIFE

Long-term safety and tolerability of ProSavin, a lentiviral vector-based gene therapy for Parkinson's disease: a dose escalation, open-label, phase 1/2 trial

(2014) Stéphane Palfi et al.   LANCET

Mesenchymal Stromal Cells Express GARP/LRRC32 on Their Surface: Effects on Their Biology and Immunomodulatory Capacity

(2014) Ana Belén Carrillo-Galvez et al.   STEM CELLS

Cord blood-derived CD34+ hematopoietic cells with low mitochondrial mass are enriched in hematopoietic repopulating stem cell function

(2013) D. Romero-Moya et al.   HAEMATOLOGICA

Lentiviral Hematopoietic Stem Cell Gene Therapy Benefits Metachromatic Leukodystrophy

(2013) A. Biffi et al.   SCIENCE

Heterogeneity of Coenzyme Q10Deficiency

(2012) Valentina Emmanuele et al.   ARCHIVES OF NEUROLOGY

nde1deletion improves mitochondrial DNA maintenance inSaccharomyces cerevisiaecoenzyme Q mutants

(2012) Fernando Gomes et al.   BIOCHEMICAL JOURNAL

Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency

(2012) L. Garcia-Corzo et al.   HUMAN MOLECULAR GENETICS

Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment

(2012) Kate E. C. Duberley et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Microscale oxygraphy reveals OXPHOS impairment in MRC mutant cells

(2012) F. Invernizzi et al.   MITOCHONDRION

Mitochondrial Complex III ROS Regulate Adipocyte Differentiation

(2011) Kathryn V. Tormos et al.   Cell Metabolism

Hematopoietic gene therapy restores thymidine phosphorylase activity in a cell culture and a murine model of MNGIE

(2011) J Torres-Torronteras et al.   GENE THERAPY

Genetic therapy for the nervous system

(2011) W. J. Bowers et al.   HUMAN MOLECULAR GENETICS

Development of an All-in-One Lentiviral Vector System Based on the Original TetR for the Easy Generation of Tet-ON Cell Lines

(2011) Karim Benabdellah et al.   PLoS One

Recent Advances in the Genetics of Mitochondrial Encephalopathies

(2010) Elena J. Tucker et al.   Current Neurology and Neuroscience Reports

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

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

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

(2010) Luis C. López et al.   PLoS One

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

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy

(2009) Ángeles Rodríguez-Hernández et al.   Autophagy

Reactive oxygen species prime Drosophila haematopoietic progenitors for differentiation

(2009) Edward Owusu-Ansah et al.   NATURE

In vivo delivery of lentiviral vectors expressing vasoactive intestinal peptide complementary DNA as gene therapy for collagen-induced arthritis

(2008) Mario Delgado et al.   ARTHRITIS AND RHEUMATISM

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

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