Loss of COX4I1 Leads to Combined Respiratory Chain Deficiency and Impaired Mitochondrial Protein Synthesis

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

COA6 Facilitates Cytochrome c Oxidase Biogenesis as Thiol-reductase for Copper Metallochaperones in Mitochondria

(2020) David Pacheu-Grau et al.   JOURNAL OF MOLECULAR BIOLOGY

Cytochrome c Oxidase Subunit 4 Isoform Exchange Results in Modulation of Oxygen Affinity

(2020) David Pajuelo Reguera et al.   Cells

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

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

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

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

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

(2020) Teresa Lobo‐Jarne et al.   EMBO JOURNAL

MITRAC15/COA1 promotes mitochondrial translation in a ND2 ribosome–nascent chain complex

(2019) Cong Wang et al.   EMBO REPORTS

COA6 Is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase

(2019) Shivatheja Soma et al.   Cell Reports

Structure of the intact 14-subunit human cytochrome c oxidase

(2018) Shuai Zong et al.   CELL RESEARCH

Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes

(2018) Alba Signes et al.   Essays in Biochemistry

Spatial orchestration of mitochondrial translation and OXPHOS complex assembly

(2018) Stefan Stoldt et al.   NATURE CELL BIOLOGY

Mitochondrial cytochrome c oxidase biogenesis: Recent developments

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

COX16 promotes COX2 metallation and assembly during respiratory complex IV biogenesis

(2018) Abhishek Aich et al.   eLife

Multi-omics analysis identifies ATF4 as a key regulator of the mitochondrial stress response in mammals

(2017) Pedro M. Quirós et al.   JOURNAL OF CELL BIOLOGY

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

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

(2017) Sara Vidoni et al.   Cell Reports

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

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

Mitochondrial Protein Synthesis Adapts to Influx of Nuclear-Encoded Protein

(2016) Ricarda Richter-Dennerlein et al.   CELL

A Genome-wide CRISPR Death Screen Identifies Genes Essential for Oxidative Phosphorylation

(2016) Jason D. Arroyo et al.   Cell Metabolism

Ground control to major TOM: mitochondria-nucleus communication

(2016) Michal Eisenberg-Bord et al.   FEBS Journal

Acadian variant of Fanconi syndrome is caused by mitochondrial respiratory chain complex I deficiency due to a non-coding mutation in complex I assembly factor NDUFAF6

(2016) Hana Hartmannová et al.   HUMAN MOLECULAR GENETICS

The architecture of respiratory supercomplexes

(2016) James A. Letts et al.   NATURE

The architecture of the mammalian respirasome

(2016) Jinke Gu et al.   NATURE

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

(2016) David A. Stroud et al.   NATURE

Human mitochondrial COX1 assembly into cytochrome c oxidase at a glance

(2015) S. Dennerlein et al.   JOURNAL OF CELL SCIENCE

Making Proteins in the Powerhouse

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

A Novel in-Frame 18-bp Microdeletion inMT-CYBCauses a Multisystem Disorder with Prominent Exercise Intolerance

(2014) Valeria Carossa et al.   HUMAN MUTATION

Double Nicking by RNA-Guided CRISPR Cas9 for Enhanced Genome Editing Specificity

(2013) F. Ann Ran et al.   CELL

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

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

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

Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core

(2011) Ileana C. Soto et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy

(2011) Maik Hüttemann et al.   MITOCHONDRION

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

Proton-Coupled Electron Transfer in Cytochrome Oxidase

(2010) Ville R. I. Kaila et al.   CHEMICAL REVIEWS

Isolation of mitochondria for biogenetical studies: An update

(2009) Erika Fernández-Vizarra et al.   MITOCHONDRION

Mitochondrial Complex III Deficiency Associated with a Homozygous Mutation in UQCRQ

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