Multiple pathways coordinate assembly of human mitochondrial complex IV and stabilization of respiratory supercomplexes

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

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

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

Mitochondrial Supercomplexes Do Not Enhance Catalysis by Quinone Channeling

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

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

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

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

Open questions: respiratory chain supercomplexes—why are they there and what do they do?

(2018) Judy Hirst   BMC BIOLOGY

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

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

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

UQCRC2 mutation in a patient with mitochondrial complex III deficiency causing recurrent liver failure, lactic acidosis and hypoglycemia

(2017) Pauline Gaignard et al.   JOURNAL OF HUMAN GENETICS

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

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

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

Structure of Mammalian Respiratory Supercomplex I 1 III 2 IV 1

(2016) Meng Wu et al.   CELL

Mitochondrial cytochrome c oxidase deficiency

(2016) M. Rak et al.   CLINICAL SCIENCE

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

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

COX7A2L/SCAFI and Pre-Complex III Modify Respiratory Chain Supercomplex Formation in Different Mouse Strains with a Bcs1l Mutation

(2016) Mina Davoudi et al.   PLoS One

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

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

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

(2015) Sven Dennerlein et al.   Cell Reports

Nuclear gene mutations as the cause of mitochondrial complex III deficiency

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

NOVA: a software to analyze complexome profiling data

(2014) H. Giese et al.   BIOINFORMATICS

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

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

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

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

Supercomplex Assembly Determines Electron Flux in the Mitochondrial Electron Transport Chain

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

A stabilizing factor for mitochondrial respiratory supercomplex assembly regulates energy metabolism in muscle

(2013) Kazuhiro Ikeda et al.   Nature Communications

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

Adaptation of respiratory chain biogenesis to cytochrome c oxidase deficiency caused by SURF1 gene mutations

(2012) Nikola Kovářová et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly

(2012) Hue-Tran Hornig-Do et al.   EMBO JOURNAL

Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity

(2011) M. A. Calvaruso et al.   HUMAN MOLECULAR GENETICS

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

Global quantification of mammalian gene expression control

(2011) Björn Schwanhäusser et al.   NATURE

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

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 of nuclear DNA-encoded subunits into mitochondrial complex IV, and their preferential integration into supercomplex forms in patient mitochondria

(2009) Michael Lazarou et al.   FEBS Journal

Development and implementation of standardized respiratory chain spectrophotometric assays for clinical diagnosis

(2009) F. Medja et al.   MITOCHONDRION

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

Respiratory Active Mitochondrial Supercomplexes

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

MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification

(2008) Jürgen Cox et al.   NATURE BIOTECHNOLOGY