Getting out what you put in: Copper in mitochondria and its impacts on human disease

Mechanisms of Mitochondrial Iron-Sulfur Protein Biogenesis

(2020) Roland Lill et al.   Annual Review of Biochemistry

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

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

From the discovery to molecular understanding of cellular iron-sulfur protein biogenesis

(2020) Roland Lill   BIOLOGICAL CHEMISTRY

20,000 picometers under the OMM : diving into the vastness of mitochondrial metabolite transport

(2020) Corey N Cunningham et al.   EMBO REPORTS

Copper is an essential regulator of the autophagic kinases ULK1/2 to drive lung adenocarcinoma

(2020) Tiffany Tsang et al.   NATURE CELL BIOLOGY

Elesclomol alleviates Menkes pathology and mortality by escorting Cu to cuproenzymes in mice

(2020) Liam M. Guthrie et al.   SCIENCE

Metallothioneins regulate ATP7A trafficking and control cell viability during copper deficiency and excess

(2020) Nikita Gudekar et al.   Scientific Reports

Diseases Caused by Mutations in Mitochondrial Carrier Genes SLC25: A Review

(2020) Ferdinando Palmieri et al.   Biomolecules

The Molecular Mechanism of Transport by the Mitochondrial ADP/ATP Carrier

(2019) Jonathan J. Ruprecht et al.   CELL

Splitting the functions of Rim2, a mitochondrial iron/pyrimidine carrier

(2019) Simon A.B. Knight et al.   MITOCHONDRION

The Role of Iron in Friedreich’s Ataxia: Insights From Studies in Human Tissues and Cellular and Animal Models

(2019) José Vicente Llorens et al.   Frontiers in Neuroscience

Mitochondrial metabolism promotes adaptation to proteotoxic stress

(2019) Peter Tsvetkov et al.   Nature Chemical Biology

Ratiometric two-photon microscopy reveals attomolar copper buffering in normal and Menkes mutant cells

(2019) M. Thomas Morgan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Trafficking mechanisms of P-type ATPase copper transporters

(2019) Cortnie Hartwig et al.   CURRENT OPINION IN CELL BIOLOGY

The Oxymonad Genome Displays Canonical Eukaryotic Complexity in the Absence of a Mitochondrion

(2019) Anna Karnkowska et al.   MOLECULAR BIOLOGY AND EVOLUTION

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

(2019) Cong Wang et al.   EMBO REPORTS

The SLC25 Mitochondrial Carrier Family: Structure and Mechanism

(2019) Jonathan J. Ruprecht et al.   TRENDS IN BIOCHEMICAL SCIENCES

Mitochondrial Iron Transporters (MIT1 and MIT2) Are Essential for Iron Homeostasis and Embryogenesis in Arabidopsis thaliana

(2019) Anshika Jain et al.   Frontiers in Plant Science

Modular biogenesis of mitochondrial respiratory complexes

(2019) Mario H. Barros et al.   MITOCHONDRION

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

Chalkophores

(2018) Grace E. Kenney et al.   Annual Review of Biochemistry

In vitroreconstitution, functional dissection, and mutational analysis of metal ion transport by mitoferrin-1

(2018) Eric T. Christenson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Copper regulates rest-activity cycles through the locus coeruleus-norepinephrine system

(2018) Tong Xiao et al.   Nature Chemical Biology

Mitochondrial cytochrome c oxidase biogenesis: Recent developments

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

Metals and Methanotrophy

(2018) Jeremy D. Semrau et al.   APPLIED AND ENVIRONMENTAL MICROBIOLOGY

Elesclomol restores mitochondrial function in genetic models of copper deficiency

(2018) Shivatheja Soma et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SFXN1 is a mitochondrial serine transporter required for one-carbon metabolism

(2018) Nora Kory et al.   SCIENCE

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

Building the CuAsite of cytochromecoxidase: A complicated, redox-dependent process driven by a surprisingly large complement of accessory proteins

(2017) Kimberly A. Jett et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The mammalian phosphate carrier SLC25A3 is a mitochondrial copper transporter required for cytochromecoxidase biogenesis

(2017) Aren Boulet et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The metal chaperone Atox1 regulates the activity of the human copper transporter ATP7B by modulating domain dynamics

(2017) Corey H. Yu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Copper signaling in the brain and beyond

(2017) Cheri M. Ackerman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Iron–sulfur cluster biogenesis and trafficking in mitochondria

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

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

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

The mitochondrion: a central architect of copper homeostasis

(2017) Zakery N. Baker et al.   Metallomics

Cryptococcus neoformans Iron-Sulfur Protein Biogenesis Machinery Is a Novel Layer of Protection against Cu Stress

(2017) Sarela Garcia-Santamarina et al.   mBio

Labile Low-Molecular-Mass Metal Complexes in Mitochondria: Trials and Tribulations of a Burgeoning Field

(2016) Paul A. Lindahl et al.   BIOCHEMISTRY

A Eukaryote without a Mitochondrial Organelle

(2016) Anna Karnkowska et al.   CURRENT BIOLOGY

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

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

A ratiometric fluorescent sensor for the mitochondrial copper pool

(2016) Clara Shen et al.   Metallomics

Copper regulates cyclic-AMP-dependent lipolysis

(2016) Lakshmi Krishnamoorthy et al.   Nature Chemical Biology

Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD

(2016) Jared R. Williams et al.   NEUROBIOLOGY OF DISEASE

Overlap of copper and iron uptake systems in mitochondria inSaccharomyces cerevisiae

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

Detection of Labile Low-Molecular-Mass Transition Metal Complexes in Mitochondria

(2015) Sean P. McCormick et al.   BIOCHEMISTRY

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

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

Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway

(2015) Sonya E. Neal et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human mitochondrial COX1 assembly into cytochrome c oxidase at a glance

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

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

Searching for harmony in transition-metal signaling

(2015) Christopher J Chang   Nature Chemical Biology

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

An integrated phylogenomic approach toward pinpointing the origin of mitochondria

(2015) Zhang Wang et al.   Scientific Reports

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

Three conserved histidine residues contribute to mitochondrial iron transport through mitoferrins

(2014) Xavier Brazzolotto et al.   BIOCHEMICAL JOURNAL

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

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

The cytotoxicity of the anticancer drug elesclomol is due to oxidative stress indirectly mediated through its complex with Cu(II)

(2014) Brian B. Hasinoff et al.   JOURNAL OF INORGANIC BIOCHEMISTRY

A chemical potentiator of copper-accumulation used to investigate the iron-regulons ofSaccharomyces cerevisiae

(2014) Andrew W. Foster et al.   MOLECULAR MICROBIOLOGY

Copper is required for oncogenic BRAF signalling and tumorigenesis

(2014) Donita C. Brady et al.   NATURE

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

The mitochondrial carrier Rim2 co-imports pyrimidine nucleotides and iron

(2013) Elisabeth M. Froschauer et al.   BIOCHEMICAL JOURNAL

SOD1 Integrates Signals from Oxygen and Glucose to Repress Respiration

(2013) Amit R. Reddi et al.   CELL

Mia40 and MINOS act in parallel with Ccs1 in the biogenesis of mitochondrial Sod1

(2013) Aksana Varabyova et al.   FEBS Journal

Golgi in copper homeostasis: a view from the membrane trafficking field

(2013) Roman Polishchuk et al.   HISTOCHEMISTRY AND CELL BIOLOGY

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

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

Review: Iron metabolism and the role of iron in neurodegenerative disorders

(2013) M. Hadzhieva et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

The Mitochondrial Pyruvate Carrier: Has It Been Unearthed at Last?

(2012) Andrew P. Halestrap   Cell Metabolism

The oncology drug elesclomol selectively transports copper to the mitochondria to induce oxidative stress in cancer cells

(2012) Masazumi Nagai et al.   FREE RADICAL BIOLOGY AND MEDICINE

A Novel Role for Copper in Ras/Mitogen-Activated Protein Kinase Signaling

(2012) M. L. Turski et al.   MOLECULAR AND CELLULAR BIOLOGY

Modular assembly of yeast cytochrome oxidase

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

Identification and Functional Expression of the Mitochondrial Pyruvate Carrier

(2012) S. Herzig et al.   SCIENCE

Rim2, a pyrimidine nucleotide exchanger, is needed for iron utilization in mitochondria

(2011) Heeyong Yoon et al.   BIOCHEMICAL JOURNAL

Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core

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

Liver mitochondrial membrane crosslinking and destruction in a rat model of Wilson disease

(2011) Hans Zischka et al.   JOURNAL OF CLINICAL INVESTIGATION

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

Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system

(2011) Dominik P. Groß et al.   MOLECULAR BIOLOGY OF THE CELL

Calcium-dependent copper redistributions in neuronal cells revealed by a fluorescent copper sensor and X-ray fluorescence microscopy

(2011) S. C. Dodani et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Copper Metallochaperones

(2010) Nigel J. Robinson et al.   Annual Review of Biochemistry

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

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

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

(2010) Jessica Garber Morales et al.   BIOCHEMISTRY

The Conserved Mitochondrial Twin Cx9C Protein Cmc2 Is a Cmc1 Homologue Essential for CytochromecOxidase Biogenesis

(2010) Darryl Horn et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Analysis of Leigh Syndrome Mutations in the Yeast SURF1 Homolog Reveals a New Member of the Cytochrome Oxidase Assembly Factor Family

(2010) M. Bestwick et al.   MOLECULAR AND CELLULAR BIOLOGY

The yeast mitochondrial carrier proteins Mrs3p/Mrs4p mediate iron transport across the inner mitochondrial membrane

(2009) Elisabeth M. Froschauer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Cytochrome c oxidase deficiency: Patients and animal models

(2009) Francisca Diaz   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

ABC transporters: the power to change

(2009) Douglas C. Rees et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

“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

Mitochondrial copper metabolism and delivery to cytochromec oxidase

(2008) Darryl Horn et al.   IUBMB LIFE

The Disulfide Relay System of Mitochondria Is Required for the Biogenesis of Mitochondrial Ccs1 and Sod1

(2008) Silvia Reddehase et al.   JOURNAL OF MOLECULAR BIOLOGY

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

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

Regulation of Mitochondrial Iron Import through Differential Turnover of Mitoferrin 1 and Mitoferrin 2

(2008) P. N. Paradkar et al.   MOLECULAR AND CELLULAR BIOLOGY

Mitochondrial copper(I) transfer from Cox17 to Sco1 is coupled to electron transfer

(2008) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The mechanism of transport by mitochondrial carriers based on analysis of symmetry

(2008) A. J. Robinson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA