Cellular mechanisms of complex I-associated pathology

Complex I and II are required for normal mitochondrial Ca2+ homeostasis

(2019) Fabian Jaña et al.   MITOCHONDRION

Human diseases associated with defects in assembly of OXPHOS complexes

(2018) Daniele Ghezzi et al.   Essays in Biochemistry

Role of mitochondrial ROS in the brain: from physiology to neurodegeneration

(2018) Plamena R. Angelova et al.   FEBS LETTERS

Impaired Bioenergetics in Mutant Mitochondrial DNA Determines Cell Fate During Seizure-Like Activity

(2018) Stjepana Kovac et al.   MOLECULAR NEUROBIOLOGY

α-synuclein oligomers interact with ATP synthase and open the permeability transition pore in Parkinson’s disease

(2018) Marthe H. R. Ludtmann et al.   Nature Communications

Mitochondrial dysfunction in Parkinsonian mesenchymal stem cells impairs differentiation

(2018) Plamena R. Angelova et al.   Redox Biology

Mammalian Mitochondrial Complex I Structure and Disease-Causing Mutations

(2018) Karol Fiedorczuk et al.   TRENDS IN CELL BIOLOGY

Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency

(2018) Guilhian Leipnitz et al.   Scientific Reports

Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD+

(2018) Eligio F. Iannetti et al.   Cell Death & Disease

The role of astrocytes in seizure generation: insights from a novel in vitro seizure model based on mitochondrial dysfunction

(2018) Felix Chan et al.   BRAIN

Reverse electron transfer results in a loss of flavin from mitochondrial complex I: Potential mechanism for brain ischemia reperfusion injury

(2017) Anna Stepanova et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Modulation of the conformational state of mitochondrial complex I as a target for therapeutic intervention

(2017) Alexander Galkin et al.   Interface Focus

Functional role of mitochondrial reactive oxygen species in physiology

(2016) Plamena R. Angelova et al.   FREE RADICAL BIOLOGY AND MEDICINE

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

(2016) Marta Kanabus et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Atomic structure of the entire mammalian mitochondrial complex I

(2016) Karol Fiedorczuk et al.   NATURE

Deletion of the Complex I Subunit NDUFS4 Adversely Modulates Cellular Differentiation

(2016) Jacqueline Johnson et al.   STEM CELLS AND DEVELOPMENT

Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency

(2016) Johannes K. Ehinger et al.   Nature Communications

Impaired Cellular Bioenergetics Causes Mitochondrial Calcium Handling Defects in MT-ND5 Mutant Cybrids

(2016) Matthew McKenzie et al.   PLoS One

Calcium and mitochondria in the regulation of cell death

(2015) Sten Orrenius et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Reduced mitochondrial Ca2+ transients stimulate autophagy in human fibroblasts carrying the 13514A>G mutation of the ND5 subunit of NADH dehydrogenase

(2015) V Granatiero et al.   CELL DEATH AND DIFFERENTIATION

Assembly defects induce oxidative stress in inherited mitochondrial complex I deficiency

(2015) Géraldine Leman et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Metabolic rescue in pluripotent cells from patients with mtDNA disease

(2015) Hong Ma et al.   NATURE

A giant molecular proton pump: structure and mechanism of respiratory complex I

(2015) Leonid A. Sazanov   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial Ca2+ in neurodegenerative disorders

(2015) Rosella Abeti et al.   PHARMACOLOGICAL RESEARCH

Aggregated α-synuclein and complex I deficiency: exploration of their relationship in differentiated neurons

(2015) A K Reeve et al.   Cell Death & Disease

Complex I dysfunction underlies the glycolytic switch in pulmonary hypertensive smooth muscle cells

(2015) Ruslan Rafikov et al.   Redox Biology

Neuronal and astrocyte dysfunction diverges from embryonic fibroblasts in theNdufs4fky/fkymouse

(2014) Matthew J. Bird et al.   BIOSCIENCE REPORTS

Soluble, Prefibrillar α-Synuclein Oligomers Promote Complex I-dependent, Ca2+-induced Mitochondrial Dysfunction

(2014) Eric S. Luth et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ischaemic accumulation of succinate controls reperfusion injury through mitochondrial ROS

(2014) Edward T. Chouchani et al.   NATURE

Tissue- and cell-type-specific manifestations of heteroplasmic mtDNA 3243A>G mutation in human induced pluripotent stem cell-derived disease model

(2013) R. H. Hamalainen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Leigh syndrome associated with mitochondrial complex I deficiency due to novel mutations In NDUFV1 and NDUFS2

(2012) Samantha E. Marin et al.   GENE

Prolonged seizure activity impairs mitochondrial bioenergetics and induces cell death

(2012) S. Kovac et al.   JOURNAL OF CELL SCIENCE

Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing

(2012) Tobias B Haack et al.   JOURNAL OF MEDICAL GENETICS

Chemiosmotic coupling in oxidative and photosynthetic phosphorylation

(2011) Peter Mitchell   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Fumonisin B1 inhibits mitochondrial respiration and deregulates calcium homeostasis—Implication to mechanism of cell toxicity

(2011) Ana-Marija Domijan et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes includingNDUFB9

(2011) Tobias B Haack et al.   JOURNAL OF MEDICAL GENETICS

Impact of fumonisin B1 on glutamate toxicity and low magnesium-induced seizure activity in neuronal primary culture

(2011) A.-M. Domijan et al.   NEUROSCIENCE

Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations

(2010) A. Y. Abramov et al.   BRAIN

Genetic variation of the mitochondrial complex I subunit NDUFV2 and Parkinson’s disease

(2010) Kenya Nishioka et al.   PARKINSONISM & RELATED DISORDERS

Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease

(2009) F. Distelmaier et al.   BRAIN

Lack of Oxygen Deactivates Mitochondrial Complex I

(2009) Alexander Galkin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rapid screening for nuclear genes mutations in isolated respiratory chain complex I defects

(2009) Hélène Pagniez-Mammeri et al.   MOLECULAR GENETICS AND METABOLISM

Respiratory Complex I Dysfunction Due to Mitochondrial DNA Mutations Shifts the Voltage Threshold for Opening of the Permeability Transition Pore toward Resting Levels

(2008) Anna Maria Porcelli et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ΔΨm-Dependent and -independent production of reactive oxygen species by rat brain mitochondria

(2003) Tatyana V. Votyakova et al.   JOURNAL OF NEUROCHEMISTRY