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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Supercomplex Assembly Determines Electron Flux in the Mitochondrial Electron Transport Chain
Esther Lapuente-Brun et al.
SCIENCE (2013)
Mitochondrial Regulation of Cell Death
Stephen W. G. Tait et al.
COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2013)
NDUFA4 Mutations Underlie Dysfunction of a Cytochrome c Oxidase Subunit Linked to Human Neurological Disease
Robert D. S. Pitceathly et al.
CELL REPORTS (2013)
Mutations in COX7B Cause Microphthalmia with Linear Skin Lesions, an Unconventional Mitochondrial Disease
Alessia Indrieri et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2012)
Mutations in C12orf62, a Factor that Couples COX I Synthesis with Cytochrome c Oxidase Assembly, Cause Fatal Neonatal Lactic Acidosis
Woranontee Weraarpachai et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2012)
Tightly-bound ubiquinone in the Escherichia coli respiratory Complex I
Michael Verkhovsky et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2012)
Biogenesis of the cytochrome bc1 complex and role of assembly factors
Pamela M. Smith et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2012)
Structure, function, and assembly of heme centers in mitochondrial respiratory complexes
Hyung J. Kim et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2012)
MITRAC Links Mitochondrial Protein Translocation to Respiratory-Chain Assembly and Translational Regulation
David U. Mick et al.
CELL (2012)
NDUFA4 Is a Subunit of Complex IV of the Mammalian Electron Transport Chain
Eduardo Balsa et al.
CELL METABOLISM (2012)
Identification of a Protein Mediating Respiratory Supercomplex Stability
Yu-Chan Chen et al.
CELL METABOLISM (2012)
Mitochondrial Complex I Plays an Essential Role in Human Respirasome Assembly
David Moreno-Lastres et al.
CELL METABOLISM (2012)
Complexome Profiling Identifies TMEM126B as a Component of the Mitochondrial Complex I Assembly Complex
Heinrich Heide et al.
CELL METABOLISM (2012)
Rcf1 Mediates Cytochrome Oxidase Assembly and Respirasome Formation, Revealing Heterogeneity of the Enzyme Complex
Milena Vukotic et al.
CELL METABOLISM (2012)
Mitochondrial Protein Synthesis, Import, and Assembly
Thomas D. Fox
GENETICS (2012)
Arrangement of the Respiratory Chain Complexes in Saccharomyces cerevisiae Supercomplex III2IV2 Revealed by Single Particle Cryo-Electron Microscopy
Eugenia Mileykovskaya et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
The Mitochondrial Oxidase Assembly Protein1 (Oxa1) Insertase Forms a Membrane Pore in Lipid Bilayers
Vivien Krueger et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
CHCM1/CHCHD6, Novel Mitochondrial Protein Linked to Regulation of Mitofilin and Mitochondrial Cristae Morphology
Jie An et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
High-resolution melting analysis of 15 genes in 60 patients with cytochrome-c oxidase deficiency
Alzbeta Vondrackova et al.
JOURNAL OF HUMAN GENETICS (2012)
Mitochondrial ATP synthase: architecture, function and pathology
An I. Jonckheere et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2012)
Defective NDUFA9 as a novel cause of neonatally fatal complex I disease
B. J. C. van den Bosch et al.
JOURNAL OF MEDICAL GENETICS (2012)
Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes including NDUFB9
Tobias B. Haack et al.
JOURNAL OF MEDICAL GENETICS (2012)
Recessive germline SDHA and SDHB mutations causing leukodystrophy and isolated mitochondrial complex II deficiency
Charlotte L. Alston et al.
JOURNAL OF MEDICAL GENETICS (2012)
Mitochondrial calcium homeostasis as potential target for mitochondrial medicine
Carlotta Giorgi et al.
MITOCHONDRION (2012)
Intermembrane Space Proteome of Yeast Mitochondria
F. -Nora Voegtle et al.
MOLECULAR & CELLULAR PROTEOMICS (2012)
Late-Stage Maturation of the Rieske Fe/S Protein: Mzm1 Stabilizes Rip1 but Does Not Facilitate Its Translocation by the AAA ATPase Bcs1
Tie-Zhong Cui et al.
MOLECULAR AND CELLULAR BIOLOGY (2012)
MELAS-like encephalomyopathy caused by a new pathogenic mutation in the mitochondrial DNA encoded cytochrome c oxidase subunit I
Costanza Lamperti et al.
NEUROMUSCULAR DISORDERS (2012)
Identification and Functional Expression of the Mitochondrial Pyruvate Carrier
Sebastien Herzig et al.
SCIENCE (2012)
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
Radek Szklarczyk et al.
GENOME BIOLOGY (2012)
A Mutation in C2orf64 Causes Impaired Cytochrome c Oxidase Assembly and Mitochondrial Cardiomyopathy
Merei Huigsloot et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2011)
A two-state stabilization-change mechanism for proton-pumping complex I
Ulrich Brandt
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2011)
Modular assembly of yeast mitochondrial ATP synthase
Malgorzata Rak et al.
EMBO JOURNAL (2011)
NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease
Saskia J. G. Hoefs et al.
EUROPEAN JOURNAL OF HUMAN GENETICS (2011)
ChChd3, an Inner Mitochondrial Membrane Protein, Is Essential for Maintaining Crista Integrity and Mitochondrial Function
Manjula Darshi et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2011)
Cbp3-Cbp6 interacts with the yeast mitochondrial ribosomal tunnel exit and promotes cytochrome b synthesis and assembly
Steffi Gruschke et al.
JOURNAL OF CELL BIOLOGY (2011)
Molecular-Clinical Correlation in a Family With a Novel Heteroplasmic Leigh Syndrome Missense Mutation in the Mitochondrial Cytochrome c Oxidase III Gene
Emna Mkaouar-Rebai et al.
JOURNAL OF CHILD NEUROLOGY (2011)
Novel single base pair COX III subunit deletion of mitochondrial DNA associated with rhabdomyolysis
Rosetta Marotta et al.
JOURNAL OF CLINICAL NEUROSCIENCE (2011)
Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome
Elsebet Ostergaard et al.
JOURNAL OF MEDICAL GENETICS (2011)
Mutations in the Gene Encoding C8orf38 Block Complex I Assembly by Inhibiting Production of the Mitochondria-Encoded Subunit ND1
Matthew McKenzie et al.
JOURNAL OF MOLECULAR BIOLOGY (2011)
A transcriptome screen in yeast identifies a novel assembly factor for the mitochondrial complex III
Lise Mathieu et al.
MITOCHONDRION (2011)
Biogenesis of mitochondrial β-barrel proteins: the POTRA domain is involved in precursor release from the SAM complex
David A. Stroud et al.
MOLECULAR BIOLOGY OF THE CELL (2011)
A Pathway of Protein Translocation in Mitochondria Mediated by the AAA-ATPase Bcsl
Nikola Wegener et al.
MOLECULAR CELL (2011)
Structure of the membrane domain of respiratory complex I
Rouslan G. Efremov et al.
NATURE (2011)
A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter
Diego De Stefani et al.
NATURE (2011)
Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies
Daniele Ghezzi et al.
NATURE GENETICS (2011)
Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations
Katherine A. Janeway et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Interaction of complexes I, III, and IV within the bovine respirasome by single particle cryoelectron tomography
Natalya V. Dudkina et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Questioning the functional relevance of mitochondrial supercomplexes by time-resolved analysis of the respiratory chain
Martin Trouillard et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Severe X-Linked Mitochondrial Encephalomyopathy Associated with a Mutation in Apoptosis-Inducing Factor
Daniele Ghezzi et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2010)
Acyl-CoA Dehydrogenase 9 Is Required for the Biogenesis of Oxidative Phosphorylation Complex I
Jessica Nouws et al.
CELL METABOLISM (2010)
Mitochondrial metabolite transport
Ferdinando Palmieri et al.
ESSAYS IN BIOCHEMISTRY: MITOCHONDRIAL FUNCTION (2010)
Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase
Aviva Levitas et al.
EUROPEAN JOURNAL OF HUMAN GENETICS (2010)
A homozygous mutation in the SCO2 gene causes a spinal muscular atrophy like presentation with stridor and respiratory insufficiency
Maciej Pronicki et al.
EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY (2010)
Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells
M. D'Aurelio et al.
HUMAN MOLECULAR GENETICS (2010)
SDHA is a tumor suppressor gene causing paraganglioma
Nelly Burnichon et al.
HUMAN MOLECULAR GENETICS (2010)
Mitochondrial ATP synthase deficiency due to a mutation in the ATP5E gene for the F1 ε subunit
Johannes A. Mayr et al.
HUMAN MOLECULAR GENETICS (2010)
Cellular Pathophysiological Consequences of BCS1L Mutations in Mitochondrial Complex III Enzyme Deficiency
Maria Moran et al.
HUMAN MUTATION (2010)
Mzm1 Influences a Labile Pool of Mitochondrial Zinc Important for Respiratory Function
Aaron Atkinson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Coa3 and Cox14 are essential for negative feedback regulation of COX1 translation in mitochondria
David U. Mick et al.
JOURNAL OF CELL BIOLOGY (2010)
MidA is a putative methyltransferase that is required for mitochondrial complex I function
Sergio Carilla-Latorre et al.
JOURNAL OF CELL SCIENCE (2010)
Extensive screening system using suspension array technology to detect mitochondrial DNA point mutations
Yutaka Nishigaki et al.
MITOCHONDRION (2010)
Succinate dehydrogenase - Assembly, regulation and role in human disease
Jared Rutter et al.
MITOCHONDRION (2010)
Exome sequencing identifies ACAD9 mutations as a cause of complex I deficiency
Tobias B. Haack et al.
NATURE GENETICS (2010)
High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency
Sarah E. Calvo et al.
NATURE GENETICS (2010)
OPA1 Mutations Associated with Dominant Optic Atrophy Influence Optic Nerve Head Size
Piero Barboni et al.
OPHTHALMOLOGY (2010)
Bioenergetic cost of making an adenosine triphosphate molecule in animal mitochondria
Ian N. Watt et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Functional Modules and Structural Basis of Conformational Coupling in Mitochondrial Complex I
Carola Hunte et al.
SCIENCE (2010)
Mutations in NDUFAF3 (C3ORF60), Encoding an NDUFAF4 (C6ORF66)-interacting Complex I Assembly Protein, Cause Fatal Neonatal Mitochondrial Disease
Ann Saada et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2009)
Exocrine Pancreatic Insufficiency, Dyserythropoeitic Anemia, and Calvarial Hyperostosis Are Caused by a Mutation in the COX4I2 Gene
Eyal Shteyer et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2009)
Identification of novel mutations in five patients with mitochondrial encephalomyopathy
Lucia Valente et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2009)
Biogenesis of the yeast cytochrome bc(1) complex
Vincenzo Zara et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2009)
Phylogenomics of the oxidative phosphorylation in fungi reveals extensive gene duplication followed by functional divergence
Marina Marcet-Houben et al.
BMC EVOLUTIONARY BIOLOGY (2009)
NOVEL MITOCHONDRIAL DNA MUTATIONS ASSOCIATED WITH CHINESE FAMILIAL HYPERTROPHIC CARDIOMYOPATHY
Yan-Ling Wei et al.
CLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY (2009)
Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus Jews
Ronen Spiegel et al.
EUROPEAN JOURNAL OF HUMAN GENETICS (2009)
Evidence that the assembly of the yeast cytochrome bc(1) complex involves the formation of a large core structure in the inner mitochondrial membrane
Vincenzo Zara et al.
FEBS JOURNAL (2009)
Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1
Scot C. Leary et al.
HUMAN MOLECULAR GENETICS (2009)
Infantile cardiomyopathy caused by a mutation in the overlapping region of mitochondrial ATPase 6 and 8 genes
S. M. Ware et al.
JOURNAL OF MEDICAL GENETICS (2009)
Functional Analysis of Yeast bcs1 Mutants Highlights the Role of Bcs1p-Specific Amino Acids in the AAA Domain
Cecile Nouet et al.
JOURNAL OF MOLECULAR BIOLOGY (2009)
Pathogenic mutations in the 5′ untranslated region of BCS1L mRNA in mitochondrial complex III deficiency
M. Carmen Gil-Borlado et al.
MITOCHONDRION (2009)
SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy
Daniele Ghezzi et al.
NATURE GENETICS (2009)
Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome
Woranontee Weraarpachai et al.
NATURE GENETICS (2009)
Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient
Alberto Blazques et al.
NEUROMUSCULAR DISORDERS (2009)
SDH5, a Gene Required for Flavination of Succinate Dehydrogenase, Is Mutated in Paraganglioma
Huai-Xiang Hao et al.
SCIENCE (2009)
NARP syndrome in a patient harbouring an insertion in the MT-ATP6 gene that results in a truncated protein
E. Lopez-Gallardo et al.
JOURNAL OF MEDICAL GENETICS (2009)
C6ORF66 is an assembly factor of mitochondrial complex
Ann Saada et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2008)
FASTKD2 nonsense mutation in an infantile mitochondrial encephalomyopathy associated with cytochrome c oxidase deficiency
Daniele Ghezzi et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2008)
Mitochondrial complex III deficiency associated with a homozygous mutation in UQCRQ
Ortal Barel et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2008)
NDUFA2 complex I mutation leads to Leigh disease
Saskia J. G. Hoefs et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2008)
Severe infantile encephalomyopathy caused by a mutation in COX6B1, a nucleus-encoded subunit of cytochrome c oxidase
Valeria Massa et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2008)
Mutation of C20orf7 Disrupts Complex I Assembly and Causes Lethal Neonatal Mitochondrial Disease
Canny Sugiana et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2008)
Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation
Itai Berger et al.
ANNALS OF NEUROLOGY (2008)
Disruption of a mitochondrial RNA-binding protein gene results in decreased cytochrome b expression and a marked reduction in ubiquinol-cytochrome c reductase activity in mouse heart mitochondria
Fenghao Xu et al.
BIOCHEMICAL JOURNAL (2008)
Structural organization of mitochondrial ATP synthase
Ilka Wittig et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2008)
A mitochondrial protein compendium elucidates complex I disease biology
David J. Pagliarini et al.
CELL (2008)
Dimer ribbons of ATP synthase shape the inner mitochondrial membrane
Mike Strauss et al.
EMBO JOURNAL (2008)
The iron-sulphur protein Ind1 is required for effective complex I assembly
Katrine Bych et al.
EMBO JOURNAL (2008)
ATP25, a new nuclear gene of Saccharomyces cerevisiae required for expression and assembly of the Atp9p subunit of mitochondrial ATPase
Xiaomei Zeng et al.
MOLECULAR BIOLOGY OF THE CELL (2008)
Respiratory Active Mitochondrial Supercomplexes
Rebeca Acin-Perez et al.
MOLECULAR CELL (2008)
TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy
Alena Cizkova et al.
NATURE GENETICS (2008)
A novel mitochondrial ATP8 gene mutation in a patient with apical hypertrophic cardiomyopathy and neuropathy
A. I. Jonckheere et al.
JOURNAL OF MEDICAL GENETICS (2008)
Sporadic Bilateral Optic Neuropathy in Children: The Role of Mitochondrial Abnormalities
Thomas M. Bosley et al.
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE (2008)
Moving mitochondria: Establishing distribution of an essential organelle
Rebecca L. Frederick et al.
TRAFFIC (2007)
Profiling phosphoproteins of yeast mitochondria reveals a role of phosphorylation in assembly of the ATP synthase
Joerg Reinders et al.
MOLECULAR & CELLULAR PROTEOMICS (2007)
Familial gastrointestinal stromal tumors and germ-line mutations
Sarah R. McWhinney et al.
NEW ENGLAND JOURNAL OF MEDICINE (2007)
Identification and characterization of cytochrome bc1 subcomplexes in mitochondria from yeast with single and double deletions of genes encoding cytochrome bc1 subunits
Vincenzo Zara et al.
FEBS JOURNAL (2007)
Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease
C. J. R. Dunning et al.
EMBO JOURNAL (2007)
Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy
Erika Fernandez-Vizarra et al.
HUMAN MOLECULAR GENETICS (2007)
A lethal defect of mitochondrial and peroxisomal fission
Hans R. Waterham et al.
NEW ENGLAND JOURNAL OF MEDICINE (2007)
Missense mutations in the BCS1L gene as a cause of the Bjornstad syndrome
J. Travis Hinson et al.
NEW ENGLAND JOURNAL OF MEDICINE (2007)
Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly
Rutger O. Vogel et al.
GENES & DEVELOPMENT (2007)
X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy
Daniel Fernandez-Moreira et al.
ANNALS OF NEUROLOGY (2007)
The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis
Scot C. Leary et al.
CELL METABOLISM (2007)
Apoptosis: A review of programmed cell death
Susan Elmore
TOXICOLOGIC PATHOLOGY (2007)
Dynamic subcompartmentalization of the mitochondrial inner membrane
Frank Vogel et al.
JOURNAL OF CELL BIOLOGY (2006)
Mitochondrial abnormalities in patients with LHON-like optic neuropathies
Khaled K. Abu-Amero et al.
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE (2006)
Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients
Matthew McKenzie et al.
JOURNAL OF MOLECULAR BIOLOGY (2006)
Origin of mitochondria by intracellular enslavement of a photosynthetic purple bacterium
Thomas Cavalier-Smith
PROCEEDINGS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES (2006)
Mitochondrial complex I: Structure, function and pathology
Rolf J. R. J. Janssen et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2006)
OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion
Christian Frezza et al.
CELL (2006)
Biosynthesis of heme in mammals
Richard S. Ajioka et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2006)
Architecture of active mammalian respiratory chain supercomplexes
Eva Schaefer et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Apoptosis-inducing factor: vital and lethal
Nazanine Modjtahedi et al.
TRENDS IN CELL BIOLOGY (2006)
Iron-sulphur clusters and the problem with oxygen
JA Imlay
MOLECULAR MICROBIOLOGY (2006)
The axonal transport of mitochondria
PJ Hollenbeck et al.
JOURNAL OF CELL SCIENCE (2005)
Taz1, an outer mitochondrial membrane protein, affects stability and assembly of inner membrane protein complexes: Implications for Barth syndrome
K Brandner et al.
MOLECULAR BIOLOGY OF THE CELL (2005)
Human mitochondrial complex I assembly is mediated by NDUFAF1
RO Vogel et al.
FEBS JOURNAL (2005)
Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency
F Diaz et al.
HUMAN MOLECULAR GENETICS (2005)
A mitochondrial cytochrome b mutation causing severe respiratory chain enzyme deficiency in humans and yeast
EL Blakely et al.
FEBS JOURNAL (2005)
Crystal structure of mitochondrial respiratory membrane protein complex II
F Sun et al.
CELL (2005)
Assembly of cytochrome-c oxidase in the absence of assembly protein Surf1p leads to loss of the active site heme
D Smith et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Saccharomyces cerevisiae translational activator Cbs1p is associated with translationally active mitochondrial ribosomes
U Krause-Buchholz et al.
BIOLOGICAL CHEMISTRY (2005)
Structure of a mitochondrial supercomplex formed by respiratory-chain complexes I and III
NV Dudkina et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
A role for Pet100p in the assembly of yeast cytochrome c oxidase -: Interaction with a subassembly that accumulates in a pet100 mutant
C Church et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Cytochrome c oxidase:: 25 years of the elusive proton pump
M Wikström
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2004)
Further insights into the assembly of the yeast cytochrome bc1 complex based on analysis of single and double deletion mutants lacking supernumerary subunits and cytochrome b
V Zara et al.
EUROPEAN JOURNAL OF BIOCHEMISTRY (2004)
Assembly of respiratory complexes I, III, and IV into NADH oxidase supercomplex stabilizes complex I in Paracoccus denitrificans
A Stroh et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
Respiratory chain complex V deficiency due to a mutation in the assembly gene ATP12
L De Meirleir et al.
JOURNAL OF MEDICAL GENETICS (2004)
An Alu-mediated partial SDHC deletion causes familial and sporadic paraganglioma
BE Baysal et al.
JOURNAL OF MEDICAL GENETICS (2004)
Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase
SC Leary et al.
HUMAN MOLECULAR GENETICS (2004)
Clinical and molecular findings in children with complex I deficiency
M Bugiani et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2004)
Electron transfer between hemes in mammalian cytochrome c oxidase
E Pilet et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2004)
OPA1 requires mitofusin 1 to promote mitochondrial fusion
S Cipolat et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2004)
Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome
P Benit et al.
JOURNAL OF MEDICAL GENETICS (2004)
De novo mutations in the mitochondrial ND3 gene as a cause of infantile mitochondrial encephalopathy and complex I deficiency
R McFarland et al.
ANNALS OF NEUROLOGY (2004)
Analysis of the composition, assembly kinetics and activity of native Apaf-1 apoptosomes
MM Hill et al.
EMBO JOURNAL (2004)
Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A
S Züchner et al.
NATURE GENETICS (2004)
Mutations of the mitochondrial ND1 gene as a cause of MELAS
DM Kirby et al.
JOURNAL OF MEDICAL GENETICS (2004)
The proteome of Saccharomyces cerevisiae mitochondria
A Sickmann et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
Mitochondrial formation of reactive oxygen species
JF Turrens
JOURNAL OF PHYSIOLOGY-LONDON (2003)
Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency
H Antonicka et al.
HUMAN MOLECULAR GENETICS (2003)
A heteroplasmic mitochondrial complex I gene mutation in adult-onset dystonia
DK Simon et al.
NEUROGENETICS (2003)
Suppressor mutations define two regions in the Cbp1 protein important for mitochondrial cytochrome b mRNA stability in Saccharomyces cerevisiae
MA Islas-Osuna et al.
CURRENT GENETICS (2003)
Machinery for protein sorting and assembly in the mitochondrial outer membrane
N Wiedemann et al.
NATURE (2003)
The cristal membrane of mitochondria is the principal site of oxidative phosphorylation
RW Gilkerson et al.
FEBS LETTERS (2003)
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophlic cardiomyopathy and encephalopathy
P Bénit et al.
HUMAN MUTATION (2003)
Protein insertion into the mitochondrial inner membrane by a twin-pore translocase
P Rehling et al.
SCIENCE (2003)
COX16 encodes a novel protein required for the assembly of cytochrome oxidase in Saccharomyces cerevisiae
CG Carlson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2003)
Identification of a gene causing human cytochrome c oxidase deficiency by integrative genomics
VK Mootha et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
Interactions among COX1, COX2, and COX3 mRNA-specific translational activator proteins on the inner surface of the mitochondrial inner membrane of Saccharomyces cerevisiae
S Naithani et al.
MOLECULAR BIOLOGY OF THE CELL (2003)
Is the mitochondrial complex IND5 gene a hot-spot for MELAS causing mutations?
D Liolitsa et al.
ANNALS OF NEUROLOGY (2003)
Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy
H Antonicka et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2003)
Childhood onset mitochondrial myopathy and lactic acidosis caused a stop mutation in the mitochondrial cytochrome c oxidase III gene
R Horváth et al.
JOURNAL OF MEDICAL GENETICS (2002)
Characterization of COX19, a widely distributed gene required for expression of mitochondrial cytochrome oxidase
MP Nobrega et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
Death and anti-death: Tumour resistance to apoptosis
FH Igney et al.
NATURE REVIEWS CANCER (2002)
Crystal structure of the yeast cytochrome bc1 complex with its bound substrate cytochrome c
C Lange et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)
The role of mtDNA background in disease expression: a new primary LHON mutation associated with Western Eurasian haplogroup J
MD Brown et al.
HUMAN GENETICS (2002)
The ATP synthase is involved in generating mitochondrial cristae morphology
P Paumard et al.
EMBO JOURNAL (2002)
In vivo regulation of oxidative phosphorylation in cells harboring a stop-codon mutation in mitochondrial DNA-encoded cytochrome c oxidase subunit I
M D'Aurelio et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Mutation spectrum and splicing variants in the OPA1 gene
C Delettre et al.
HUMAN GENETICS (2001)
Multisystem disorder associated with a missense mutation in the mitochondrial cytochrome b gene
F Wibrand et al.
ANNALS OF NEUROLOGY (2001)
An mtDNA mutation, 14453G→A, in the NADH dehydrogenase subunit 6 associated with severe MELAS syndrome
K Ravn et al.
EUROPEAN JOURNAL OF HUMAN GENETICS (2001)
A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure
P de Lonlay et al.
NATURE GENETICS (2001)
Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma
D Astuti et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2001)
Spectrum, frequency and penetrance of OPA1 mutations in dominant optic atrophy
C Toomes et al.
HUMAN MOLECULAR GENETICS (2001)
OPA1 mutations in patients with autosomal dominant optic atrophy and evidence for semi-dominant inheritance
UEA Pesch et al.
HUMAN MOLECULAR GENETICS (2001)
Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency
P Bénit et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2001)
Reconstitution of mitochondrial processing peptidase from the core proteins (subunits I and II) of bovine heart mitochondrial cytochrome bc1 complex
K Deng et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Clinical spectrum and diagnosis of mitochondrial disorders
A Munnich et al.
AMERICAN JOURNAL OF MEDICAL GENETICS (2001)
Mitochondrial encephalomyopathy and complex III deficiency associated with a stop-codon mutation in the cytochrome b gene
JA Keightley et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2000)
A missense mutation in the mitochondrial cytochrome b gene in a revisited case with histiocytoid cardiomyopathy
AL Andreu et al.
PEDIATRIC RESEARCH (2000)
Combined enzymatic complex I and III deficiency associated with mutations in the nuclear encoded NDUFS4 gene
SMS Budde et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2000)
Cloning and characterization of COX18, a Saccharomyces cerevisiae PET gene required for the assembly of cytochrome oxidase
RL Souza et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2000)
Supercomplexes in the respiratory chains of yeast and mammalian mitochondria
H Schägger et al.
EMBO JOURNAL (2000)
Identification of Cox20p, a novel protein involved in the maturation and assembly of cytochrome oxidase subunit 2
K Hell et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2000)
Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma
BE Baysal et al.
SCIENCE (2000)
Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome
B Parfait et al.
HUMAN GENETICS (2000)
Cox11p is required for stable formation of the CuB and magnesium centers of cytochrome c oxidase
L Hiser et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2000)
The renaissance of mitochondrial calcium transport
T Pozzan et al.
EUROPEAN JOURNAL OF BIOCHEMISTRY (2000)
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