Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes

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

Oxygen Activation and Energy Conservation by Cytochrome c Oxidase

(2018) Mårten Wikström et al.   CHEMICAL REVIEWS

Human diseases associated with defects in assembly of OXPHOS complexes

(2018) Daniele Ghezzi et al.   Essays in Biochemistry

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

Respiratory chain supercomplexes: Structures, function and biogenesis

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

Building a complex complex: Assembly of mitochondrial respiratory chain complex I

(2018) Luke E. Formosa et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

COX16 promotes COX2 metallation and assembly during respiratory complex IV biogenesis

(2018) Abhishek Aich et al.   eLife

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

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

Iron–sulfur cluster biogenesis and trafficking in mitochondria

(2017) Joseph J. Braymer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Biogenesis and functions of mammalian iron-sulfur proteins in the regulation of iron homeostasis and pivotal metabolic pathways

(2017) Tracey A. Rouault et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Permeability transition in human mitochondria persists in the absence of peripheral stalk subunits of ATP synthase

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

Regulation of Mammalian 13-Subunit Cytochrome c Oxidase and Binding of other Proteins: Role of NDUFA4

(2017) Bernhard Kadenbach   TRENDS IN ENDOCRINOLOGY AND METABOLISM

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

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

Structure of Mammalian Respiratory Supercomplex I 1 III 2 IV 1

(2016) Meng Wu et al.   CELL

Mitochondrial Protein Synthesis Adapts to Influx of Nuclear-Encoded Protein

(2016) Ricarda Richter-Dennerlein et al.   CELL

The process of mammalian mitochondrial protein synthesis

(2016) Nicole Mai 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

Respiratory supercomplexes and the functional segmentation of the CoQ pool

(2016) Raquel Moreno-Loshuertos et al.   FREE RADICAL BIOLOGY AND MEDICINE

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

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

Exome sequencing coupled with mRNA analysis identifies NDUFAF6 as a Leigh gene

(2016) Laura Bianciardi et al.   MOLECULAR GENETICS AND METABOLISM

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

Structure of mammalian respiratory complex I

(2016) Jiapeng Zhu et al.   NATURE

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

(2016) David A. Stroud et al.   NATURE

Regulatory role of the respiratory supercomplex factors inSaccharomyces cerevisiae

(2016) Camilla Rydström Lundin 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

Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice

(2016) Tara R. Richman et al.   Nature Communications

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

Functional asymmetry and electron flow in the bovine respirasome

(2016) Joana S Sousa et al.   eLife

The dimerization of the yeast cytochrome bc1 complex is an early event and is independent of Rip1

(2015) Annalea Conte et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

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

Assembly of human mitochondrial ATP synthase through two separate intermediates, F1-c-ring andb-e-gcomplex

(2015) Makoto Fujikawa et al.   FEBS LETTERS

Mitochondrial disease genesCOA6,COX6BandSCO2have overlapping roles in COX2 biogenesis

(2015) Alok Ghosh 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

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

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

(2015) Sven Dennerlein et al.   Cell Reports

Structure and conformational states of the bovine mitochondrial ATP synthase by cryo-EM

(2015) Anna Zhou et al.   eLife

Nuclear gene mutations as the cause of mitochondrial complex III deficiency

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

The LYR Factors SDHAF1 and SDHAF3 Mediate Maturation of the Iron-Sulfur Subunit of Succinate Dehydrogenase

(2014) Un Na et al.   Cell Metabolism

SDHAF4 Promotes Mitochondrial Succinate Dehydrogenase Activity and Prevents Neurodegeneration

(2014) Jonathan G. Van Vranken et al.   Cell Metabolism

The Respiratory Chain Supercomplex Organization Is Independent of COX7a2l Isoforms

(2014) Arnaud Mourier et al.   Cell Metabolism

Cochaperone Binding to LYR Motifs Confers Specificity of Iron Sulfur Cluster Delivery

(2014) Nunziata Maio 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

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

The arginine methyltransferase NDUFAF7 is essential for complex I assembly and early vertebrate embryogenesis

(2014) Olga Zurita Rendón et al.   HUMAN MOLECULAR GENETICS

Assembly factors monitor sequential hemylation of cytochromebto regulate mitochondrial translation

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

TMEM70 deficiency: long-term outcome of 48 patients

(2014) Martin Magner et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Architecture of mammalian respiratory complex I

(2014) Kutti R. Vinothkumar et al.   NATURE

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

(2013) John E. Walker   BIOCHEMICAL SOCIETY TRANSACTIONS

The function of the respiratory supercomplexes: The plasticity model

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

I Function, Therefore I Am: Overcoming Skepticism about Mitochondrial Supercomplexes

(2013) Antoni Barrientos et al.   Cell Metabolism

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

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

Crystal structure of the entire respiratory complex I

(2013) Rozbeh Baradaran et al.   NATURE

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

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 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

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

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

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

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

Early complex I assembly defects result in rapid turnover of the ND1 subunit

(2012) O. Zurita Rendon et al.   HUMAN MOLECULAR GENETICS

Flavinylation and Assembly of Succinate Dehydrogenase Are Dependent on the C-terminal Tail of the Flavoprotein Subunit

(2012) Hyung J. Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Cbp3–Cbp6 complex coordinates cytochromebsynthesis withbc1complex assembly in yeast mitochondria

(2012) Steffi Gruschke et al.   JOURNAL OF CELL BIOLOGY

Rcf1 and Rcf2, Members of the Hypoxia-Induced Gene 1 Protein Family, Are Critical Components of the Mitochondrial Cytochrome bc1-Cytochrome c Oxidase Supercomplex

(2012) V. Strogolova et al.   MOLECULAR AND CELLULAR BIOLOGY

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

Modular assembly of yeast cytochrome oxidase

(2012) Gavin P. McStay et al.   MOLECULAR BIOLOGY OF THE CELL

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

Biogenesis of the cytochrome bc1 complex and role of assembly factors

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

Understanding mitochondrial complex I assembly in health and disease

(2011) Masakazu Mimaki et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

(2011) Benedetta Ruzzenente 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

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

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

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

(2011) Nikola Wagener et al.   MOLECULAR CELL

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

(2011) Daniele Ghezzi et al.   NATURE GENETICS

Novel insights into the assembly and function of human nuclear-encoded cytochromecoxidase subunits 4, 5a, 6a, 7a and 7b

(2010) Daniela Fornuskova et al.   BIOCHEMICAL JOURNAL

Assembly and oligomerization of human ATP synthase lacking mitochondrial subunits a and A6L

(2010) Ilka Wittig et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

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

(2010) Jessica Nouws et al.   Cell Metabolism

FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy

(2010) Elisa Fassone et al.   HUMAN MOLECULAR GENETICS

Yeast Models of Human Mitochondrial Diseases

(2010) Antoni Barrientos   IUBMB LIFE

The architecture of respiratory complex I

(2010) Rouslan G. Efremov et al.   NATURE

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

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

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

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

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

Mutations in the heme b-binding residue of SDHC inhibit assembly of respiratory chain complex II in mammalian cells

(2009) Anthony Lemarie 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

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

Mitochondrial Complex III Deficiency Associated with a Homozygous Mutation in UQCRQ

(2008) Ortal Barel 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

Structural organization of mitochondrial ATP synthase

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

The Q-cycle reviewed: How well does a monomeric mechanism of the bc1 complex account for the function of a dimeric complex?

(2008) Antony R. Crofts et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Assembly of the oxidative phosphorylation system in humans: What we have learned by studying its defects

(2008) Erika Fernández-Vizarra et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Biogenesis of the yeast cytochrome bc1 complex

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

Assembly of mitochondrial complex I and defects in disease

(2008) Michael Lazarou 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

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