Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency

Mutations in NDUFAF3 (C3ORF60), Encoding an NDUFAF4 (C6ORF66)-Interacting Complex I Assembly Protein, Cause Fatal Neonatal Mitochondrial Disease

(2009) Ann Saada et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Architecture of complex I and its implications for electron transfer and proton pumping

(2009) Volker Zickermann et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial medicine: entering the era of treatment

(2009) S. Koene et al.   JOURNAL OF INTERNAL MEDICINE

The antioxidant Trolox restores mitochondrial membrane potential and Ca2+-stimulated ATP production in human complex I deficiency

(2009) Felix Distelmaier et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement

(2009) E. Leshinsky-Silver et al.   MOLECULAR GENETICS AND METABOLISM

NDUFA2 Complex I Mutation Leads to Leigh Disease

(2008) Saskia J.G. Hoefs et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Inherited complex I deficiency is associated with faster protein diffusion in the matrix of moving mitochondria

(2008) Werner J. H. Koopman et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Mitochondrial function and morphology are impaired inparkin-mutant fibroblasts

(2008) Heather Mortiboys et al.   ANNALS OF NEUROLOGY

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Mitigation of NADH: Ubiquinone oxidoreductase deficiency by chronic Trolox treatment

(2008) Werner J.H. Koopman et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Targeting lipophilic cations to mitochondria

(2008) Michael P. Murphy   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Assembly of mitochondrial complex I and defects in disease

(2008) Michael Lazarou et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency☆

(2008) P WILLEMS et al.   CELL CALCIUM

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism

The Sirtuin family: therapeutic targets to treat diseases of aging

(2008) Jill C Milne et al.   CURRENT OPINION IN CHEMICAL BIOLOGY

The Variability of the Harlequin Mouse Phenotype Resembles that of Human Mitochondrial-Complex I-Deficiency Syndromes

(2008) Paule Bénit et al.   PLoS One

Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat

(2008) W.-S. Choi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA