Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions

Timing of dimerization of the bc complex during mitochondrial respiratory chain assembly

(2020) Katharina Stephan et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Respiratory supercomplexes act as a platform for complex III ‐mediated maturation of human mitochondrial complexes I and IV

(2020) Margherita Protasoni et al.   EMBO JOURNAL

Mutations in FASTKD2 are associated with mitochondrial disease with multi‐OXPHOS deficiency

(2020) Xiujuan Wei et al.   HUMAN MUTATION

Structures of AAA protein translocase Bcs1 suggest translocation mechanism of a folded protein

(2020) Wai Kwan Tang et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Structure of the Bcs1 AAA-ATPase suggests an airlock-like translocation mechanism for folded proteins

(2020) Lukas Kater et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Rcf1 Modulates Cytochrome c Oxidase Activity Especially Under Energy-Demanding Conditions

(2020) Hannah Dawitz et al.   Frontiers in Physiology

HIGD2A is required for assembly of the COX3 module of human mitochondrial complex IV

(2020) Daniella H Hock et al.   MOLECULAR & CELLULAR PROTEOMICS

Rcf2 revealed in cryo-EM structures of hypoxic isoforms of mature mitochondrial III-IV supercomplexes

(2020) Andrew M. Hartley et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I

(2020) Luke E. Formosa et al.   Cell Reports

Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis

(2020) Alba Timón-Gómez et al.   Cell Reports

TMEM70 functions in the assembly of complexes I and V

(2020) Laura Sánchez-Caballero et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Bi‐allelic pathogenic variants in NDUFC2 cause early‐onset Leigh syndrome and stalled biogenesis of complex I

(2020) Ahmad Alahmad et al.   EMBO Molecular Medicine

Cytochrome c oxidase deficiency

(2020) Michele Brischigliaro et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The yeast mitochondrial proteins Rcf1 and Rcf2 support the enzymology of the cytochrome c oxidase complex and generation of the proton motive force

(2019) Vera Strogolova et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial transport serves as a mitochondrial quality control strategy in axons: Implications for central nervous system disorders

(2019) Yan‐Rong Zheng et al.   CNS Neuroscience & Therapeutics

Structure and Mechanisms of F-Type ATP Synthases

(2019) Werner Kühlbrandt   Annual Review of Biochemistry

COX6A2 variants cause a muscle‐specific cytochrome c oxidase deficiency

(2019) Michio Inoue et al.   ANNALS OF NEUROLOGY

Molecular genetic investigations identify new clinical phenotypes associated with BCS1L-related mitochondrial disease

(2019) Monika Oláhová et al.   HUMAN MOLECULAR GENETICS

Structures of Respiratory Supercomplex I+III2 Reveal Functional and Conformational Crosstalk

(2019) James A. Letts et al.   MOLECULAR CELL

A Novel TTC19 Mutation in a Patient With Neurological, Psychological, and Gastrointestinal Impairment

(2019) Parham Habibzadeh et al.   Frontiers in Neurology

Pathogenic Bi-allelic Mutations in NDUFAF8 Cause Leigh Syndrome with an Isolated Complex I Deficiency

(2019) Charlotte L. Alston et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Bi-Allelic UQCRFS1 Variants Are Associated with Mitochondrial Complex III Deficiency, Cardiomyopathy, and Alopecia Totalis

(2019) Mirjana Gusic et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Molecular Wiring of a Mitochondrial Translational Feedback Loop

(2019) Roger Salvatori et al.   MOLECULAR CELL

Novel compound heterozygous pathogenic variants in nucleotide-binding protein like protein (NUBPL) cause leukoencephalopathy with multi-systemic involvement

(2019) Margherita Protasoni et al.   MOLECULAR GENETICS AND METABOLISM

NDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy

(2018) Dorota Piekutowska-Abramczuk et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Biallelic Mutations in ATP5F1D , which Encodes a Subunit of ATP Synthase, Cause a Metabolic Disorder

(2018) Monika Oláhová et al.   AMERICAN JOURNAL OF HUMAN GENETICS

COX16 is required for assembly of cytochrome c oxidase in human cells and is involved in copper delivery to COX2

(2018) Cristina Cerqua et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis

(2018) Isotta Lorenzi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

SCO2 mutations cause early-onset axonal Charcot-Marie-Tooth disease associated with cellular copper deficiency

(2018) Adriana P Rebelo et al.   BRAIN

Mitochondrial complex III Rieske Fe-S protein processing and assembly

(2018) Erika Fernandez-Vizarra et al.   CELL CYCLE

Mitochondrial Supercomplexes Do Not Enhance Catalysis by Quinone Channeling

(2018) Justin G. Fedor et al.   Cell Metabolism

Structure of the intact 14-subunit human cytochrome c oxidase

(2018) Shuai Zong et al.   CELL RESEARCH

Mitophagy and Quality Control Mechanisms in Mitochondrial Maintenance

(2018) Sarah Pickles et al.   CURRENT BIOLOGY

Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes

(2018) Alba Signes et al.   Essays in Biochemistry

Human diseases associated with defects in assembly of OXPHOS complexes

(2018) Daniele Ghezzi et al.   Essays in Biochemistry

Mitochondrial dynamics: overview of molecular mechanisms

(2018) Lisa Tilokani et al.   Essays in Biochemistry

The Complexity of Mitochondrial Complex IV: An Update of Cytochrome c Oxidase Biogenesis in Plants

(2018) et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Biogenesis of the bc 1 Complex of the Mitochondrial Respiratory Chain

(2018) Mama Ndi et al.   JOURNAL OF MOLECULAR BIOLOGY

Mitochondrial β-oxidation of saturated fatty acids in humans

(2018) María M. Adeva-Andany et al.   MITOCHONDRION

Spatial orchestration of mitochondrial translation and OXPHOS complex assembly

(2018) Stefan Stoldt et al.   NATURE CELL BIOLOGY

Conserved in situ arrangement of complex I and III2in mitochondrial respiratory chain supercomplexes of mammals, yeast, and plants

(2018) Karen M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Assembly of the membrane domain of ATP synthase in human mitochondria

(2018) Jiuya He et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial cytochrome c oxidase biogenesis: Recent developments

(2018) Alba Timón-Gómez et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Respiratory chain supercomplexes: Structures, function and biogenesis

(2018) Teresa Lobo-Jarne et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

COX16 promotes COX2 metallation and assembly during respiratory complex IV biogenesis

(2018) Abhishek Aich et al.   eLife

Biallelic Mutations in NDUFA6 Establish Its Role in Early-Onset Isolated Mitochondrial Complex I Deficiency

(2018) Charlotte L. Alston et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A new era for electron bifurcation

(2018) John W Peters et al.   CURRENT OPINION IN CHEMICAL BIOLOGY

Clinical spectrum of BCS1L Mitopathies and their underlying structural relationships

(2018) Rachael A. Baker et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS

(2018) Alba Signes et al.   EMBO Molecular Medicine

Diverse phenotype in patients with complex I deficiency due to mutations in NDUFB11

(2018) Karit Reinson et al.   European Journal of Medical Genetics

Structure of yeast cytochrome c oxidase in a supercomplex with cytochrome bc1

(2018) Andrew M. Hartley et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Cryo-EM structure of the yeast respiratory supercomplex

(2018) Sorbhi Rathore et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Locking loop movement in the ubiquinone pocket of complex I disengages the proton pumps

(2018) Alfredo Cabrera-Orefice et al.   Nature Communications

Human COX7A2L Regulates Complex III Biogenesis and Promotes Supercomplex Organization Remodeling without Affecting Mitochondrial Bioenergetics

(2018) Teresa Lobo-Jarne et al.   Cell Reports

The Mitochondrion as Potential Interface in Early-Life Stress Brain Programming

(2018) Anke Hoffmann et al.   Frontiers in Behavioral Neuroscience

Mutation in the COX4I1 gene is associated with short stature, poor weight gain and increased chromosomal breaks, simulating Fanconi anemia

(2017) Bassam Abu-Libdeh et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Mutated PET117 causes complex IV deficiency and is associated with neurodevelopmental regression and medulla oblongata lesions

(2017) G. H. Renkema et al.   HUMAN GENETICS

Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive

(2017) Fabian Baertling et al.   HUMAN MUTATION

Human mitochondrial cytochromecoxidase assembly factor COX18 acts transiently as a membrane insertase within the subunit 2 maturation module

(2017) Myriam Bourens et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The constriction and scission machineries involved in mitochondrial fission

(2017) Felix Kraus et al.   JOURNAL OF CELL SCIENCE

TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III

(2017) Emanuela Bottani et al.   MOLECULAR CELL

Clarifying the supercomplex: the higher-order organization of the mitochondrial electron transport chain

(2017) James A Letts et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Tissue- and Condition-Specific Isoforms of Mammalian Cytochrome c Oxidase Subunits: From Function to Human Disease

(2017) Christopher A. Sinkler et al.   Oxidative Medicine and Cellular Longevity

Combined Respiratory Chain Deficiency and UQCC2 Mutations in Neonatal Encephalomyopathy: Defective Supercomplex Assembly in Complex III Deficiencies

(2017) René G. Feichtinger et al.   Oxidative Medicine and Cellular Longevity

Crystal structure of CO-bound cytochromecoxidase determined by serial femtosecond X-ray crystallography at room temperature

(2017) Izumi Ishigami et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Persistence of the mitochondrial permeability transition in the absence of subunit c of human ATP synthase

(2017) Jiuya He et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Genetic diagnosis of Mendelian disorders via RNA sequencing

(2017) Laura S. Kremer et al.   Nature Communications

Clarifying the supercomplex: the higher-order organization of the mitochondrial electron transport chain

(2017) James A Letts et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

MR-1S Interacts with PET100 and PET117 in Module-Based Assembly of Human Cytochrome c Oxidase

(2017) Sara Vidoni et al.   Cell Reports

Lifetime imaging of GFP at CoxVIIIa reports respiratory supercomplex assembly in live cells

(2017) Bettina Rieger et al.   Scientific Reports

Mutations in Complex I Assembly Factor TMEM126B Result in Muscle Weakness and Isolated Complex I Deficiency

(2016) Laura Sánchez-Caballero et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype

(2016) Charlotte L. Alston et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Maintenance and Expression of Mammalian Mitochondrial DNA

(2016) Claes M. Gustafsson et al.   Annual Review of Biochemistry

Assembly of the Escherichia coli NADH:ubiquinone oxidoreductase (respiratory complex I)

(2016) Thorsten Friedrich et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Energy conversion, redox catalysis and generation of reactive oxygen species by respiratory complex I

(2016) Judy Hirst et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Unraveling the complexity of mitochondrial complex I assembly: A dynamic process

(2016) Laura Sánchez-Caballero et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Evolution and structural organization of the mitochondrial contact site (MICOS) complex and the mitochondrial intermembrane space bridging (MIB) complex

(2016) Martijn A. Huynen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

LYRM7mutations cause a multifocal cavitating leukoencephalopathy with distinct MRI appearance

(2016) Cristina Dallabona et al.   BRAIN

The TIM23 mitochondrial protein import complex: function and dysfunction

(2016) Keren Demishtein-Zohary et al.   CELL AND TISSUE RESEARCH

Disease-Causing SDHAF1 Mutations Impair Transfer of Fe-S Clusters to SDHB

(2016) Nunziata Maio et al.   Cell Metabolism

Bax assembly into rings and arcs in apoptotic mitochondria is linked to membrane pores

(2016) R. Salvador-Gallego et al.   EMBO JOURNAL

Bax assembles into large ring-like structures remodeling the mitochondrial outer membrane in apoptosis

(2016) L. Grosse et al.   EMBO JOURNAL

Mutations in the accessory subunitNDUFB10result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly

(2016) Marisa W. Friederich et al.   HUMAN MOLECULAR GENETICS

The Assembly Factor Pet117 Couples Heme a Synthase Activity to Cytochrome Oxidase Assembly

(2016) Nicholas G. Taylor et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

NDUFAF5 Hydroxylates NDUFS7 at an Early Stage in the Assembly of Human Complex I

(2016) Virginie F. Rhein et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

COA7(C1orf163/RESA1) mutations associated with mitochondrial leukoencephalopathy and cytochrome c oxidase deficiency

(2016) Anabel Martinez Lyons et al.   JOURNAL OF MEDICAL GENETICS

A Mutation in the Flavin Adenine Dinucleotide-Dependent Oxidoreductase FOXRED1 Results in Cell-Type-Specific Assembly Defects in Oxidative Phosphorylation Complexes I and II

(2016) Olga Zurita Rendón et al.   MOLECULAR AND CELLULAR BIOLOGY

The architecture of respiratory supercomplexes

(2016) James A. Letts et al.   NATURE

The architecture of the mammalian respirasome

(2016) Jinke Gu et al.   NATURE

Mechanism of super-assembly of respiratory complexes III and IV

(2016) Sara Cogliati et al.   NATURE

Accessory subunits are integral for assembly and function of human mitochondrial complex I

(2016) David A. Stroud et al.   NATURE

Complex I assembly into supercomplexes determines differential mitochondrial ROS production in neurons and astrocytes

(2016) Irene Lopez-Fabuel et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Systems proteomics of liver mitochondria function

(2016) E. G. Williams et al.   SCIENCE

Mitochondrial Cristae: Where Beauty Meets Functionality

(2016) Sara Cogliati et al.   TRENDS IN BIOCHEMICAL SCIENCES

The CoQH2/CoQ Ratio Serves as a Sensor of Respiratory Chain Efficiency

(2016) Adela Guarás et al.   Cell Reports

Optic Atrophy 1 Is Epistatic to the Core MICOS Component MIC60 in Mitochondrial Cristae Shape Control

(2016) Christina Glytsou et al.   Cell Reports

COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation

(2016) Rafael Pérez-Pérez et al.   Cell Reports

The MIA Pathway: A Key Regulator of Mitochondrial Oxidative Protein Folding and Biogenesis

(2015) Amelia Mordas et al.   ACCOUNTS OF CHEMICAL RESEARCH

Mutations in NDUFB11, Encoding a Complex I Component of the Mitochondrial Respiratory Chain, Cause Microphthalmia with Linear Skin Defects Syndrome

(2015) Vanessa A. van Rahden et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Phenotypic variation ofTTC19-deficient mitochondrial complex III deficiency: A case report and literature review

(2015) Dylan A. Mordaunt et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

A novel mutation MT-COIII m.9267G>C and MT-COI m.5913G>A mutation in mitochondrial genes in a Tunisian family with maternally inherited diabetes and deafness (MIDD) associated with sever nephropathy

(2015) Mouna Tabebi et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Iron –sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery

(2015) Nunziata Maio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Structure and function of mitochondrial membrane protein complexes

(2015) Werner Kühlbrandt   BMC BIOLOGY

Loss of the smallest subunit of cytochromecoxidase, COX8A, causes Leigh-like syndrome and epilepsy

(2015) Kerstin Hallmann et al.   BRAIN

Mic10 Oligomerizes to Bend Mitochondrial Inner Membranes at Cristae Junctions

(2015) Mariam Barbot et al.   Cell Metabolism

Cooperation between COA6 and SCO2 in COX2 Maturation during Cytochrome c Oxidase Assembly Links Two Mitochondrial Cardiomyopathies

(2015) David Pacheu-Grau et al.   Cell Metabolism

Opa1 Overexpression Ameliorates the Phenotype of Two Mitochondrial Disease Mouse Models

(2015) Gabriele Civiletto et al.   Cell Metabolism

Reaction Mechanism of Cytochrome c Oxidase

(2015) Shinya Yoshikawa et al.   CHEMICAL REVIEWS

Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability

(2015) Claire Angebault et al.   HUMAN MOLECULAR GENETICS

COA6 is a mitochondrial complex IV assembly factor critical for biogenesis of mtDNA-encoded COX2

(2015) David A. Stroud et al.   HUMAN MOLECULAR GENETICS

Characterization of mitochondrial FOXRED1 in the assembly of respiratory chain complex I

(2015) Luke E. Formosa et al.   HUMAN MOLECULAR GENETICS

The subunit composition and function of mammalian cytochrome c oxidase

(2015) Bernhard Kadenbach et al.   MITOCHONDRION

Redox-regulated dynamic interplay between Cox19 and the copper-binding protein Cox11 in the intermembrane space of mitochondria facilitates biogenesis of cytochrome c oxidase

(2015) Manuela Bode et al.   MOLECULAR BIOLOGY OF THE CELL

Higd1ais a positive regulator of cytochromecoxidase

(2015) Takaharu Hayashi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Organization of Mitochondrial Gene Expression in Two Distinct Ribosome-Containing Assemblies

(2015) Kirsten Kehrein et al.   Cell Reports

MITRAC7 Acts as a COX1-Specific Chaperone and Reveals a Checkpoint during Cytochrome c Oxidase Assembly

(2015) Sven Dennerlein et al.   Cell Reports

Mitochondrial RNA Granules Are Centers for Posttranscriptional RNA Processing and Ribosome Biogenesis

(2015) Hana Antonicka et al.   Cell Reports

Trading Places—Switching Frataxin Function by a Single Amino Acid Substitution within the [Fe-S] Cluster Assembly Scaffold

(2015) Dennis R. Dean et al.   PLoS Genetics

QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology

(2015) Virginia Guarani et al.   eLife

Nuclear gene mutations as the cause of mitochondrial complex III deficiency

(2015) Erika Fernández-Vizarra et al.   Frontiers in Genetics

A Mutation of COX6A1 Causes a Recessive Axonal or Mixed Form of Charcot-Marie-Tooth Disease

(2014) Gen Tamiya et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations in APOPT1, Encoding a Mitochondrial Protein, Cause Cavitating Leukoencephalopathy with Cytochrome c Oxidase Deficiency

(2014) Laura Melchionda et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A Founder Mutation in PET100 Causes Isolated Complex IV Deficiency in Lebanese Individuals with Leigh Syndrome

(2014) Sze Chern Lim et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Single-molecule in vivo imaging of bacterial respiratory complexes indicates delocalized oxidative phosphorylation

(2014) Isabel Llorente-Garcia et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Exome sequencing reveals a novel TTC19 mutation in an autosomal recessive spinocerebellar ataxia patient

(2014) Hiroyuki Morino et al.   BMC Neurology

The Respiratory Chain Supercomplex Organization Is Independent of COX7a2l Isoforms

(2014) Arnaud Mourier et al.   Cell Metabolism

Making Proteins in the Powerhouse

(2014) B. Martin Hällberg et al.   Cell Metabolism

Protein-mediated assembly of succinate dehydrogenase and its cofactors

(2014) Jonathan G. Van Vranken et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

The INA complex facilitates assembly of the peripheral stalk of the mitochondrial F1Fo-ATP synthase

(2014) O. Lytovchenko et al.   EMBO JOURNAL

A truncating PET100 variant causing fatal infantile lactic acidosis and isolated cytochrome c oxidase deficiency

(2014) Monika Oláhová et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

A mutation in the human CBP4 ortholog UQCC3 impairs complex III assembly, activity and cytochrome b stability

(2014) Bas F.J. Wanschers et al.   HUMAN MOLECULAR GENETICS

Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase

(2014) Myriam Bourens et al.   HUMAN MOLECULAR GENETICS

Mutations inCOA6cause CytochromecOxidase Deficiency and Neonatal Hypertrophic Cardiomyopathy

(2014) Fabian Baertling et al.   HUMAN MUTATION

An update on complex I assembly: the assembly of players

(2014) Rasika S. Vartak et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Uniform nomenclature for the mitochondrial contact site and cristae organizing system

(2014) Nikolaus Pfanner et al.   JOURNAL OF CELL BIOLOGY

Assembly factors monitor sequential hemylation of cytochromebto regulate mitochondrial translation

(2014) Markus Hildenbeutel et al.   JOURNAL OF CELL BIOLOGY

Mitochondrial disease associated with complex I (NADH-CoQ oxidoreductase) deficiency

(2014) Immo E. Scheffler   JOURNAL OF INHERITED METABOLIC DISEASE

Architecture of mammalian respiratory complex I

(2014) Kutti R. Vinothkumar et al.   NATURE

Electrostatics, hydration, and proton transfer dynamics in the membrane domain of respiratory complex I

(2014) V. R. I. Kaila et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Kinetic evidence against partitioning of the ubiquinone pool and the catalytic relevance of respiratory-chain supercomplexes

(2014) J. N. Blaza et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Heme in pathophysiology: a matter of scavenging, metabolism and trafficking across cell membranes

(2014) Deborah Chiabrando et al.   Frontiers in Pharmacology

Mutations in CYC1, Encoding Cytochrome c1 Subunit of Respiratory Chain Complex III, Cause Insulin-Responsive Hyperglycemia

(2013) Pauline Gaignard et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial Respiratory Supercomplex Association Limits Production of Reactive Oxygen Species from Complex I

(2013) Evelina Maranzana et al.   ANTIOXIDANTS & REDOX SIGNALING

The ATP synthase: the understood, the uncertain and the unknown

(2013) John E. Walker   BIOCHEMICAL SOCIETY TRANSACTIONS

Unanswered questions about the structure of cytochrome bc1 complexes

(2013) Edward A. Berry et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Iron/sulfur proteins biogenesis in prokaryotes: Formation, regulation and diversity

(2013) Béatrice Roche et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The function of the respiratory supercomplexes: The plasticity model

(2013) Rebeca Acin-Perez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

A complex V ATP5A1 defect causes fatal neonatal mitochondrial encephalopathy

(2013) An I. Jonckheere et al.   BRAIN

Mitochondrial genetics

(2013) P. F. Chinnery et al.   BRITISH MEDICAL BULLETIN

Mitochondrial Cristae Shape Determines Respiratory Chain Supercomplexes Assembly and Respiratory Efficiency

(2013) Sara Cogliati et al.   CELL

A Homozygous Mutation inLYRM7/MZM1LAssociated with Early Onset Encephalopathy, Lactic Acidosis, and Severe Reduction of Mitochondrial Complex III Activity

(2013) Federica Invernizzi et al.   HUMAN MUTATION

Post-translational Modifications near the Quinone Binding Site of Mammalian Complex I

(2013) Joe Carroll et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

hCOA3 Stabilizes CytochromecOxidase 1 (COX1) and Promotes CytochromecOxidase Assembly in Human Mitochondria

(2013) Paula Clemente et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

NDUFAF7 Methylates Arginine 85 in the NDUFS2 Subunit of Human Complex I

(2013) Virginie F. Rhein et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial lipid transport at a glance

(2013) M. Scharwey et al.   JOURNAL OF CELL SCIENCE

Novel mutation in AAA domain of BCS1L causing Bjornstad syndrome

(2013) Saima Siddiqi et al.   JOURNAL OF HUMAN GENETICS

TIMMDC1/C3orf1 Functions as a Membrane-Embedded Mitochondrial Complex I Assembly Factor through Association with the MCIA Complex

(2013) Virginia Guarani et al.   MOLECULAR AND CELLULAR BIOLOGY

Novel TTC19 mutation in a family with severe psychiatric manifestations and complex III deficiency

(2013) Célia Nogueira et al.   NEUROGENETICS

SURF1 deficiency causes demyelinating Charcot-Marie-Tooth disease

(2013) A. Echaniz-Laguna et al.   NEUROLOGY

Targeted exome sequencing of suspected mitochondrial disorders

(2013) D. S. Lieber et al.   NEUROLOGY

Assembly factors for the membrane arm of human complex I

(2013) B. Andrews et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Supercomplex Assembly Determines Electron Flux in the Mitochondrial Electron Transport Chain

(2013) E. Lapuente-Brun et al.   SCIENCE

Mitochondrial Regulation of Cell Death

(2013) S. W. G. Tait et al.   Cold Spring Harbor Perspectives in Biology

NDUFA4 Mutations Underlie Dysfunction of a Cytochrome c Oxidase Subunit Linked to Human Neurological Disease

(2013) Robert D.S. Pitceathly et al.   Cell Reports

Mutations in the UQCC1-Interacting Protein, UQCC2, Cause Human Complex III Deficiency Associated with Perturbed Cytochrome b Protein Expression

(2013) Elena J. Tucker et al.   PLoS Genetics

Mutations in COX7B Cause Microphthalmia with Linear Skin Lesions, an Unconventional Mitochondrial Disease

(2012) Alessia Indrieri et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations in C12orf62, a Factor that Couples COX I Synthesis with Cytochrome c Oxidase Assembly, Cause Fatal Neonatal Lactic Acidosis

(2012) Woranontee Weraarpachai et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Tightly-bound ubiquinone in the Escherichia coli respiratory Complex I

(2012) Michael Verkhovsky et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells

(2012) Ester Sánchez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Catalytic mechanisms of complex II enzymes: A structural perspective

(2012) T.M. Iverson   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Structure, function, and assembly of heme centers in mitochondrial respiratory complexes

(2012) Hyung J. Kim et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

MITRAC Links Mitochondrial Protein Translocation to Respiratory-Chain Assembly and Translational Regulation

(2012) David U. Mick et al.   CELL

NDUFA4 Is a Subunit of Complex IV of the Mammalian Electron Transport Chain

(2012) Eduardo Balsa et al.   Cell Metabolism

Identification of a Protein Mediating Respiratory Supercomplex Stability

(2012) Yu-Chan Chen et al.   Cell Metabolism

Mitochondrial Complex I Plays an Essential Role in Human Respirasome Assembly

(2012) David Moreno-Lastres et al.   Cell Metabolism

Complexome Profiling Identifies TMEM126B as a Component of the Mitochondrial Complex I Assembly Complex

(2012) Heinrich Heide et al.   Cell Metabolism

Rcf1 Mediates Cytochrome Oxidase Assembly and Respirasome Formation, Revealing Heterogeneity of the Enzyme Complex

(2012) Milena Vukotic et al.   Cell Metabolism

Mitochondrial Protein Synthesis, Import, and Assembly

(2012) Thomas D. Fox   GENETICS

A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia

(2012) Radek Szklarczyk et al.   HUMAN MOLECULAR GENETICS

Mitochondrial Complex III Deficiency Caused by a HomozygousUQCRC2Mutation Presenting with Neonatal-Onset Recurrent Metabolic Decompensation

(2012) Noriko Miyake et al.   HUMAN MUTATION

ATP synthase oligomerization: From the enzyme models to the mitochondrial morphology

(2012) Johan Habersetzer et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Arrangement of the Respiratory Chain Complexes inSaccharomyces cerevisiaeSupercomplex III2IV2Revealed by Single Particle Cryo-Electron Microscopy

(2012) Eugenia Mileykovskaya et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Mitochondrial Oxidase Assembly Protein1 (Oxa1) Insertase Forms a Membrane Pore in Lipid Bilayers

(2012) Vivien Krüger et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CHCM1/CHCHD6, Novel Mitochondrial Protein Linked to Regulation of Mitofilin and Mitochondrial Cristae Morphology

(2012) Jie An et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

High-resolution melting analysis of 15 genes in 60 patients with cytochrome-c oxidase deficiency

(2012) Alzbeta Vondrackova et al.   JOURNAL OF HUMAN GENETICS

Recessive germlineSDHAandSDHBmutations causing leukodystrophy and isolated mitochondrial complex II deficiency

(2012) Charlotte L Alston et al.   JOURNAL OF MEDICAL GENETICS

Intermembrane Space Proteome of Yeast Mitochondria

(2012) F.-Nora Vögtle et al.   MOLECULAR & CELLULAR PROTEOMICS

Late-Stage Maturation of the Rieske Fe/S Protein: Mzm1 Stabilizes Rip1 but Does Not Facilitate Its Translocation by the AAA ATPase Bcs1

(2012) T.-Z. Cui et al.   MOLECULAR AND CELLULAR BIOLOGY

MELAS-like encephalomyopathy caused by a new pathogenic mutation in the mitochondrial DNA encoded cytochrome c oxidase subunit I

(2012) Costanza Lamperti et al.   NEUROMUSCULAR DISORDERS

Identification and Functional Expression of the Mitochondrial Pyruvate Carrier

(2012) S. Herzig et al.   SCIENCE

Iterative orthology prediction uncovers new mitochondrial proteins and identifies C12orf62 as the human ortholog of COX14, a protein involved in the assembly of cytochrome c oxidase

(2012) Radek Szklarczyk et al.   GENOME BIOLOGY

A Mutation in C2orf64 Causes Impaired Cytochrome c Oxidase Assembly and Mitochondrial Cardiomyopathy

(2011) Merei Huigsloot et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A two-state stabilization-change mechanism for proton-pumping complex I

(2011) Ulrich Brandt   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Biogenesis of the cytochrome bc1 complex and role of assembly factors

(2011) Pamela M. Smith et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Modular assembly of yeast mitochondrial ATP synthase

(2011) Malgorzata Rak et al.   EMBO JOURNAL

Cbp3–Cbp6 interacts with the yeast mitochondrial ribosomal tunnel exit and promotes cytochromebsynthesis and assembly

(2011) Steffi Gruschke et al.   JOURNAL OF CELL BIOLOGY

Mitochondrial ATP synthase: architecture, function and pathology

(2011) An I. Jonckheere et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Defective NDUFA9 as a novel cause of neonatally fatal complex I disease

(2011) B J C van den Bosch et al.   JOURNAL OF MEDICAL GENETICS

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

Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome

(2011) E. Ostergaard et al.   JOURNAL OF MEDICAL GENETICS

Mutations in the Gene Encoding C8orf38 Block Complex I Assembly by Inhibiting Production of the Mitochondria-Encoded Subunit ND1

(2011) Matthew McKenzie et al.   JOURNAL OF MOLECULAR BIOLOGY

Mitochondrial calcium homeostasis as potential target for mitochondrial medicine

(2011) Carlotta Giorgi et al.   MITOCHONDRION

The LYR Protein Mzm1 Functions in the Insertion of the Rieske Fe/S Protein in Yeast Mitochondria

(2011) A. Atkinson et al.   MOLECULAR AND CELLULAR BIOLOGY

Cells Lacking Rieske Iron-Sulfur Protein Have a Reactive Oxygen Species-Associated Decrease in Respiratory Complexes I and IV

(2011) F. Diaz et al.   MOLECULAR AND CELLULAR BIOLOGY

Biogenesis of mitochondrial β-barrel proteins: the POTRA domain is involved in precursor release from the SAM complex

(2011) David A. Stroud et al.   MOLECULAR BIOLOGY OF THE CELL

A Pathway of Protein Translocation in Mitochondria Mediated by the AAA-ATPase Bcs1

(2011) Nikola Wagener et al.   MOLECULAR CELL

Structure of the membrane domain of respiratory complex I

(2011) Rouslan G. Efremov et al.   NATURE

A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter

(2011) Diego De Stefani et al.   NATURE

Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies

(2011) Daniele Ghezzi et al.   NATURE GENETICS

Interaction of complexes I, III, and IV within the bovine respirasome by single particle cryoelectron tomography

(2011) N. V. Dudkina et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Questioning the functional relevance of mitochondrial supercomplexes by time-resolved analysis of the respiratory chain

(2011) M. Trouillard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Severe X-Linked Mitochondrial Encephalomyopathy Associated with a Mutation in Apoptosis-Inducing Factor

(2010) Daniele Ghezzi et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Acyl-CoA Dehydrogenase 9 Is Required for the Biogenesis of Oxidative Phosphorylation Complex I

(2010) Jessica Nouws et al.   Cell Metabolism

Mitochondrial metabolite transport

(2010) Ferdinando Palmieri et al.   Essays in Biochemistry

NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease

(2010) Saskia J G Hoefs et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase

(2010) Aviva Levitas et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

SDHA is a tumor suppressor gene causing paraganglioma

(2010) Nelly Burnichon et al.   HUMAN MOLECULAR GENETICS

Mitochondrial ATP synthase deficiency due to a mutation in the ATP5E gene for the F1   subunit

(2010) J. A. Mayr et al.   HUMAN MOLECULAR GENETICS

Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency

(2010) María Morán et al.   HUMAN MUTATION

ChChd3, an Inner Mitochondrial Membrane Protein, Is Essential for Maintaining Crista Integrity and Mitochondrial Function

(2010) Manjula Darshi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mzm1 Influences a Labile Pool of Mitochondrial Zinc Important for Respiratory Function

(2010) Aaron Atkinson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Coa3 and Cox14 are essential for negative feedback regulation ofCOX1translation in mitochondria

(2010) David U. Mick et al.   JOURNAL OF CELL BIOLOGY

MidA is a putative methyltransferase that is required for mitochondrial complex I function

(2010) S. Carilla-Latorre et al.   JOURNAL OF CELL SCIENCE

Molecular-Clinical Correlation in a Family With a Novel Heteroplasmic Leigh Syndrome Missense Mutation in the Mitochondrial Cytochrome c Oxidase III Gene

(2010) Emna Mkaouar-Rebai et al.   JOURNAL OF CHILD NEUROLOGY

Novel single base pair COX III subunit deletion of mitochondrial DNA associated with rhabdomyolysis

(2010) Rosetta Marotta et al.   JOURNAL OF CLINICAL NEUROSCIENCE

Extensive screening system using suspension array technology to detect mitochondrial DNA point mutations

(2010) Yutaka Nishigaki et al.   MITOCHONDRION

Succinate dehydrogenase – Assembly, regulation and role in human disease

(2010) Jared Rutter et al.   MITOCHONDRION

A transcriptome screen in yeast identifies a novel assembly factor for the mitochondrial complex III

(2010) Lise Mathieu et al.   MITOCHONDRION

Exome sequencing identifies ACAD9 mutations as a cause of complex I deficiency

(2010) Tobias B Haack et al.   NATURE GENETICS

High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency

(2010) Sarah E Calvo et al.   NATURE GENETICS

OPA1 Mutations Associated with Dominant Optic Atrophy Influence Optic Nerve Head Size

(2010) Piero Barboni et al.   OPHTHALMOLOGY

Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations

(2010) K. A. Janeway et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Bioenergetic cost of making an adenosine triphosphate molecule in animal mitochondria

(2010) I. N. Watt et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Functional Modules and Structural Basis of Conformational Coupling in Mitochondrial Complex I

(2010) C. Hunte et al.   SCIENCE

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

Exocrine Pancreatic Insufficiency, Dyserythropoeitic Anemia, and Calvarial Hyperostosis Are Caused by a Mutation in the COX4I2 Gene

(2009) Eyal Shteyer et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Phylogenomics of the oxidative phosphorylation in fungi reveals extensive gene duplication followed by functional divergence

(2009) Marina Marcet-Houben et al.   BMC EVOLUTIONARY BIOLOGY

NOVEL MITOCHONDRIAL DNA MUTATIONS ASSOCIATED WITH CHINESE FAMILIAL HYPERTROPHIC CARDIOMYOPATHY

(2009) Yan-Ling Wei et al.   CLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY

Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus Jews

(2009) Ronen Spiegel et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

A homozygous mutation in the SCO2 gene causes a spinal muscular atrophy like presentation with stridor and respiratory insufficiency

(2009) Maciej Pronicki et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Evidence that the assembly of the yeast cytochrome bc1 complex involves the formation of a large core structure in the inner mitochondrial membrane

(2009) Vincenzo Zara et al.   FEBS Journal

Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells

(2009) M. D'Aurelio et al.   HUMAN MOLECULAR GENETICS

Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1

(2009) Scot C. Leary et al.   HUMAN MOLECULAR GENETICS

Infantile cardiomyopathy caused by a mutation in the overlapping region of mitochondrial ATPase 6 and 8 genes

(2009) S M Ware et al.   JOURNAL OF MEDICAL GENETICS

Functional Analysis of Yeast bcs1 Mutants Highlights the Role of Bcs1p-Specific Amino Acids in the AAA Domain

(2009) Cécile Nouet et al.   JOURNAL OF MOLECULAR BIOLOGY

Pathogenic mutations in the 5′ untranslated region of BCS1L mRNA in mitochondrial complex III deficiency

(2009) M. Carmen Gil-Borlado et al.   MITOCHONDRION

Human Ind1, an Iron-Sulfur Cluster Assembly Factor for Respiratory Complex I

(2009) A. D. Sheftel et al.   MOLECULAR AND CELLULAR BIOLOGY

SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy

(2009) Daniele Ghezzi et al.   NATURE GENETICS

Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

(2009) Woranontee Weraarpachai et al.   NATURE GENETICS

Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient

(2009) Alberto Blázquez et al.   NEUROMUSCULAR DISORDERS

SDH5, a Gene Required for Flavination of Succinate Dehydrogenase, Is Mutated in Paraganglioma

(2009) H.-X. Hao et al.   SCIENCE

C6ORF66 Is an Assembly Factor of Mitochondrial Complex I

(2008) Ann Saada et al.   AMERICAN JOURNAL OF HUMAN GENETICS

FASTKD2 Nonsense Mutation in an Infantile Mitochondrial Encephalomyopathy Associated with Cytochrome C Oxidase Deficiency

(2008) Daniele Ghezzi et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial Complex III Deficiency Associated with a Homozygous Mutation in UQCRQ

(2008) Ortal Barel et al.   AMERICAN JOURNAL OF HUMAN GENETICS

NDUFA2 Complex I Mutation Leads to Leigh Disease

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

Severe Infantile Encephalomyopathy Caused by a Mutation in COX6B1, a Nucleus-Encoded Subunit of Cytochrome C Oxidase

(2008) Valeria Massa et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutation of C20orf7 Disrupts Complex I Assembly and Causes Lethal Neonatal Mitochondrial Disease

(2008) Canny Sugiana et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation

(2008) Itai Berger et al.   ANNALS OF NEUROLOGY

Disruption of a mitochondrial RNA-binding protein gene results in decreased cytochromebexpression and a marked reduction in ubiquinol–cytochromecreductase activity in mouse heart mitochondria

(2008) Fenghao Xu et al.   BIOCHEMICAL JOURNAL

Structural organization of mitochondrial ATP synthase

(2008) Ilka Wittig et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Identification of novel mutations in five patients with mitochondrial encephalomyopathy

(2008) Lucia Valente et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Biogenesis of the yeast cytochrome bc1 complex

(2008) Vincenzo Zara et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

Dimer ribbons of ATP synthase shape the inner mitochondrial membrane

(2008) Mike Strauss et al.   EMBO JOURNAL

The iron–sulphur protein Ind1 is required for effective complex I assembly

(2008) Katrine Bych et al.   EMBO JOURNAL

ATP25, a New Nuclear Gene of Saccharomyces cerevisiae Required for Expression and Assembly of the Atp9p Subunit of Mitochondrial ATPase

(2008) Xiaomei Zeng et al.   MOLECULAR BIOLOGY OF THE CELL

Respiratory Active Mitochondrial Supercomplexes

(2008) Rebeca Acín-Pérez et al.   MOLECULAR CELL

TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy

(2008) Alena Čížková et al.   NATURE GENETICS