The mitochondrion: a central architect of copper homeostasis

The mitochondrial metallochaperone SCO1 maintains CTR1 at the plasma membrane to preserve copper homeostasis in the murine heart

(2017) Zakery N Baker et al.   HUMAN MOLECULAR GENETICS

[4Fe-4S] Cluster Assembly in Mitochondria and Its Impairment by Copper

(2017) Diego Brancaccio et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Oxygen-dependent activation of Cu,Zn-superoxide dismutase-1

(2017) Morgan M. Fetherolf et al.   Metallomics

Mitochondrial Sirtuin Network Reveals Dynamic SIRT3-Dependent Deacetylation in Response to Membrane Depolarization

(2016) Wen Yang et al.   CELL

The coming of age of the mitochondria–ER contact: a matter of thickness

(2016) M Giacomello et al.   CELL DEATH AND DIFFERENTIATION

The Activity of Menkes Disease Protein ATP7A Is Essential for Redox Balance in Mitochondria

(2016) Ashima Bhattacharjee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Syntaxin-17 delivers PINK1/parkin-dependent mitochondrial vesicles to the endolysosomal system

(2016) Gian-Luca McLelland et al.   JOURNAL OF CELL BIOLOGY

Methanobactin reverses acute liver failure in a rat model of Wilson disease

(2016) Josef Lichtmannegger et al.   JOURNAL OF CLINICAL INVESTIGATION

Formation of mitochondrial-derived vesicles is an active and physiologically relevant mitochondrial quality control process in the cardiac system

(2016) Virgilio J. J. Cadete et al.   JOURNAL OF PHYSIOLOGY-LONDON

Copper regulates cyclic-AMP-dependent lipolysis

(2016) Lakshmi Krishnamoorthy et al.   Nature Chemical Biology

Neuronal differentiation is associated with a redox-regulated increase of copper flow to the secretory pathway

(2016) Yuta Hatori et al.   Nature Communications

Overlap of copper and iron uptake systems in mitochondria inSaccharomyces cerevisiae

(2016) Katherine E. Vest et al.   Open Biology

Thiol switches in mitochondria: operation and physiological relevance

(2015) Jan Riemer et al.   BIOLOGICAL CHEMISTRY

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

Evolution of Mitochondria as Signaling Organelles

(2015) Navdeep S. Chandel   Cell Metabolism

Redox Homeostasis and Mitochondrial Dynamics

(2015) Peter H.G.M. Willems et al.   Cell Metabolism

The mitochondria–plasma membrane contact site

(2015) Benedikt Westermann   CURRENT OPINION IN CELL BIOLOGY

Cardiac deficiency of single cytochrome oxidase assembly factor scox induces p53-dependent apoptosis in a Drosophila cardiomyopathy model

(2015) Leticia Martínez-Morentin et al.   HUMAN MOLECULAR GENETICS

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

Human mitochondrial COX1 assembly into cytochrome c oxidase at a glance

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

Kinetic control by limiting glutaredoxin amounts enables thiol oxidation in the reducing mitochondrial intermembrane space

(2015) Kerstin Kojer et al.   MOLECULAR BIOLOGY OF THE CELL

A cytosolic network suppressing mitochondria-mediated proteostatic stress and cell death

(2015) Xiaowen Wang et al.   NATURE

Mistargeted mitochondrial proteins activate a proteostatic response in the cytosol

(2015) Lidia Wrobel et al.   NATURE

Searching for harmony in transition-metal signaling

(2015) Christopher J Chang   Nature Chemical Biology

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

Loop recognition and copper-mediated disulfide reduction underpin metal site assembly of CuAin human cytochrome oxidase

(2015) Marcos N. Morgada et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Mitochondrial Metallochaperone SCO1 Is Required to Sustain Expression of the High-Affinity Copper Transporter CTR1 and Preserve Copper Homeostasis

(2015) Christopher J. Hlynialuk et al.   Cell Reports

Impact of copper deficiency in humans

(2014) Joseph R. Prohaska   Annals of the New York Academy of Sciences

Pathological mitochondrial copper overload in livers of Wilson's disease patients and related animal models

(2014) Hans Zischka et al.   Annals of the New York Academy of Sciences

Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency

(2014) A. Ghosh et al.   HUMAN MOLECULAR GENETICS

Mutations inCOA6cause CytochromecOxidase Deficiency and Neonatal Hypertrophic Cardiomyopathy

(2014) Fabian Baertling et al.   HUMAN MUTATION

SIRT3 regulates cellular iron metabolism and cancer growth by repressing iron regulatory protein 1

(2014) S M Jeong et al.   ONCOGENE

Copper is an endogenous modulator of neural circuit spontaneous activity

(2014) Sheel C. Dodani et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cellular glutathione plays a key role in copper uptake mediated by human copper transporter 1

(2013) Edward B. Maryon et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Novel Mutations inSCO1as a Cause of Fatal Infantile Encephalopathy and Lactic Acidosis

(2013) Scot C. Leary et al.   HUMAN MUTATION

Copper Import into the Mitochondrial Matrix in Saccharomyces cerevisiae Is Mediated by Pic2, a Mitochondrial Carrier Family Protein

(2013) Katherine E. Vest et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The natural history of SCO2 deficiency in 36 Polish children confirmed the genotype–phenotype correlation

(2013) Ewa Pronicka et al.   MITOCHONDRION

COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux

(2013) Scot C. Leary et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial disulfide relay mediates translocation of p53 and partitions its subcellular activity

(2013) J. Zhuang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Charting the travels of copper in eukaryotes from yeast to mammals

(2012) Tracy Nevitt et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The intracellular redox state is a core determinant of mitochondrial fusion

(2012) Timothy Shutt et al.   EMBO REPORTS

Functional Partnership of the Copper Export Machinery and Glutathione Balance in Human Cells

(2012) Yuta Hatori et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Fusion and Fission: Interlinked Processes Critical for Mitochondrial Health

(2011) David C. Chan   Annual Review of Genetics

Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core

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

Mitochondrial Disulfide Relay: Redox-regulated Protein Import into the Intermembrane Space

(2011) Johannes M. Herrmann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Targetable Fluorescent Sensor Reveals That Copper-DeficientSCO1andSCO2Patient Cells Prioritize Mitochondrial Copper Homeostasis

(2011) Sheel C. Dodani et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol

(2011) Christine Klöppel et al.   MOLECULAR BIOLOGY OF THE CELL

Advances in metal-induced oxidative stress and human disease

(2011) Klaudia Jomova et al.   TOXICOLOGY

Import, Maturation, and Function of SOD1 and Its Copper Chaperone CCS in the Mitochondrial Intermembrane Space

(2010) Hibiki Kawamata et al.   ANTIOXIDANTS & REDOX SIGNALING

Redox Regulation of SCO Protein Function: Controlling Copper at a Mitochondrial Crossroad

(2010) Scot C. Leary   ANTIOXIDANTS & REDOX SIGNALING

Biophysical Characterization of Iron in Mitochondria Isolated from Respiring and Fermenting Yeast

(2010) Jessica Garber Morales et al.   BIOCHEMISTRY

Role of Glutaredoxin1 and Glutathione in Regulating the Activity of the Copper-transporting P-type ATPases, ATP7A and ATP7B

(2010) William C. J. Singleton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Affinity gradients drive copper to cellular destinations

(2010) Lucia Banci et al.   NATURE

Loss of function of Sco1 and its interaction with cytochrome c oxidase

(2009) Lukas Stiburek et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Mitochondrial Glutathione, a Key Survival Antioxidant

(2009) Montserrat Marí et al.   ANTIOXIDANTS & REDOX SIGNALING

Analysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2

(2009) Hua Yang 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

Systematic Analysis of the Twin Cx9C Protein Family

(2009) Sebastian Longen et al.   JOURNAL OF MOLECULAR BIOLOGY

Dysregulation of intracellular copper trafficking pathway in a mouse model of mutant copper/zinc superoxide dismutase-linked familial amyotrophic lateral sclerosis

(2009) Eiichi Tokuda et al.   JOURNAL OF NEUROCHEMISTRY

Three-dimensional structure of the human copper transporter hCTR1

(2009) C. J. De Feo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The iron-sulfur clusters of dehydratases are primary intracellular targets of copper toxicity

(2009) L. Macomber et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Disulfide Formation in the ER and Mitochondria: Two Solutions to a Common Process

(2009) J. Riemer et al.   SCIENCE

“Pulling the plug” on cellular copper: The role of mitochondria in copper export

(2008) Scot C. Leary et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Cargo-Selected Transport from the Mitochondria to Peroxisomes Is Mediated by Vesicular Carriers

(2008) Margaret Neuspiel et al.   CURRENT BIOLOGY

Deleterious Role of Superoxide Dismutase in the Mitochondrial Intermembrane Space

(2008) Gundars Goldsteins et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cmc1p Is a Conserved Mitochondrial Twin CX9C Protein Involved in Cytochrome c Oxidase Biogenesis

(2008) D. Horn et al.   MOLECULAR AND CELLULAR BIOLOGY

Mechanisms for copper acquisition, distribution and regulation

(2008) Byung-Eun Kim et al.   Nature Chemical Biology

Mitochondrial structure and function in the untreated Jackson toxic milk (tx-j) mouse, a model for Wilson disease

(2007) Eve A. Roberts et al.   MOLECULAR GENETICS AND METABOLISM