CHCHD4 (MIA40) and the mitochondrial disulfide relay system

AIF meets the CHCHD4/Mia40-dependent mitochondrial import pathway

(2020) Camille Reinhardt et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Protein import by the mitochondrial disulfide relay in higher eukaryotes

(2020) Yannik Finger et al.   BIOLOGICAL CHEMISTRY

A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency

(2020) Yukiko Yatsuka et al.   CLINICAL GENETICS

The C-terminal region of the oxidoreductase MIA40 stabilizes its cytosolic precursor during mitochondrial import

(2020) Lena Maria Murschall et al.   BMC BIOLOGY

Mitochondrial Metabolism as a Target for Cancer Therapy

(2020) Karthik Vasan et al.   Cell Metabolism

Apoptosis inducing factor and mitochondrial NADH dehydrogenases: redox-controlled gear boxes to switch between mitochondrial biogenesis and cell death

(2020) Johannes M. Herrmann et al.   BIOLOGICAL CHEMISTRY

Proteasomal degradation induced by DPP9‐mediated processing competes with mitochondrial protein import

(2020) Yannik Finger et al.   EMBO JOURNAL

An antibody toolbox to track complex I assembly defines AIF’s mitochondrial function

(2020) Anjaneyulu Murari et al.   JOURNAL OF CELL BIOLOGY

Glutathionylated and Fe–S cluster containing hMIA40 (CHCHD4) regulates ROS and mitochondrial complex III and IV activities of the electron transport chain

(2020) Venkata Ramana Thiriveedi et al.   Redox Biology

Vectorial Import via a Metastable Disulfide-Linked Complex Allows for a Quality Control Step and Import by the Mitochondrial Disulfide Relay

(2019) Markus Habich et al.   Cell Reports

Development of the Mitochondrial Intermembrane Space Disulfide Relay Represents a Critical Step in Eukaryotic Evolution

(2019) Sandra Backes et al.   MOLECULAR BIOLOGY AND EVOLUTION

Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7

(2019) Karthik Mohanraj et al.   EMBO Molecular Medicine

AIF-regulated oxidative phosphorylation supports lung cancer development

(2019) Shuan Rao et al.   CELL RESEARCH

mTORC1 as a Regulator of Mitochondrial Functions and a Therapeutic Target in Cancer

(2019) Karen Griselda de la Cruz López et al.   Frontiers in Oncology

A novel CHCHD10 mutation implicates a Mia40‐dependent mitochondrial import deficit in ALS

(2018) Carina Lehmer et al.   EMBO Molecular Medicine

New insights into the mechanisms of epithelial–mesenchymal transition and implications for cancer

(2018) Anushka Dongre et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Loss of the Mia40a oxidoreductase leads to hepato-pancreatic insufficiency in zebrafish

(2018) Anna M. Sokol et al.   PLoS Genetics

VHL-Mediated Regulation of CHCHD4 and Mitochondrial Function

(2018) Thomas Briston et al.   Frontiers in Oncology

Iron–sulfur cluster biogenesis and trafficking in mitochondria

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

Osm1 facilitates the transfer of electrons from Erv1 to fumarate in the redox-regulated import pathway in the mitochondrial intermembrane space

(2017) Sonya E. Neal et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondria: back to the future

(2017) Navdeep S. Chandel   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The mTORC1 Signaling Network Senses Changes in Cellular Purine Nucleotide Levels

(2017) Gerta Hoxhaj et al.   Cell Reports

CHCHD4 Regulates Intracellular Oxygenation and Perinuclear Distribution of Mitochondria

(2017) Luke W. Thomas et al.   Frontiers in Oncology

Mitochondrial disulfide relay and its substrates: mechanisms in health and disease

(2016) Alican J. Erdogan et al.   CELL AND TISSUE RESEARCH

Mutations in the accessory subunitNDUFB10result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly

(2016) Marisa W. Friederich et al.   HUMAN MOLECULAR GENETICS

A disulfide bond in the TIM23 complex is crucial for voltage gating and mitochondrial protein import

(2016) Ajay Ramesh et al.   JOURNAL OF CELL BIOLOGY

COA7(C1orf163/RESA1) mutations associated with mitochondrial leukoencephalopathy and cytochrome c oxidase deficiency

(2016) Anabel Martinez Lyons et al.   JOURNAL OF MEDICAL GENETICS

Structure/Function Relations in AIFM1 Variants Associated with Neurodegenerative Disorders

(2016) Irina F. Sevrioukova   JOURNAL OF MOLECULAR BIOLOGY

Structure of mammalian respiratory complex I

(2016) Jiapeng Zhu et al.   NATURE

The presence of disulfide bonds reveals an evolutionarily conserved mechanism involved in mitochondrial protein translocase assembly

(2016) Lidia Wrobel et al.   Scientific Reports

Mia40 is a trans-site receptor that drives protein import into the mitochondrial intermembrane space by hydrophobic substrate binding

(2016) Valentina Peleh et al.   eLife

Human mitochondrial MIA40 (CHCHD4) is a component of the Fe-S cluster export machinery

(2015) A. Murari et al.   BIOCHEMICAL JOURNAL

The roles of glutaredoxins ligating Fe–S clusters: Sensing, transfer or repair functions?

(2015) Jérémy Couturier et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The Ca2+-Dependent Release of the Mia40-Induced MICU1-MICU2 Dimer from MCU Regulates Mitochondrial Ca2+ Uptake

(2015) Carmelina Petrungaro et al.   Cell Metabolism

Liver-Specific Deletion of Augmenter of Liver Regeneration Accelerates Development of Steatohepatitis and Hepatocellular Carcinoma in Mice

(2015) Chandrashekhar R. Gandhi et al.   GASTROENTEROLOGY

Adrenal Insufficiency in Mitochondrial Disease

(2015) Laurel Calderwood et al.   JOURNAL OF CHILD NEUROLOGY

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

Interaction between AIF and CHCHD4 Regulates Respiratory Chain Biogenesis

(2015) Emilie Hangen et al.   MOLECULAR CELL

Loss of apoptosis-inducing factor critically affects MIA40 function

(2015) K Meyer et al.   Cell Death & Disease

ROS Function in Redox Signaling and Oxidative Stress

(2014) Michael Schieber et al.   CURRENT BIOLOGY

Targeting tumour hypoxia to prevent cancer metastasis. From biology, biosensing and technology to drug development: the METOXIA consortium

(2014) Erik O. Pettersen et al.   JOURNAL OF ENZYME INHIBITION AND MEDICINAL CHEMISTRY

MICU1 and MICU2 Finely Tune the Mitochondrial Ca2+ Uniporter by Exerting Opposite Effects on MCU Activity

(2014) Maria Patron et al.   MOLECULAR CELL

Visualization of Redox-Controlled Protein Fold in Living Cells

(2013) Lucia Banci et al.   CHEMISTRY & BIOLOGY

The Disulfide Relay of the Intermembrane Space Oxidizes the Ribosomal Subunit Mrp10 on Its Transit into the Mitochondrial Matrix

(2013) Sebastian Longen et al.   DEVELOPMENTAL CELL

C1orf163/RESA1 Is a Novel Mitochondrial Intermembrane Space Protein Connected to Respiratory Chain Assembly

(2013) Vera Kozjak-Pavlovic et al.   JOURNAL OF MOLECULAR BIOLOGY

Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells

(2013) Manuel Fischer et al.   MOLECULAR BIOLOGY OF THE CELL

Atp23 biogenesis reveals a chaperone-like folding activity of Mia40 in the IMS of mitochondria

(2012) Daniel Weckbecker et al.   EMBO JOURNAL

Cytosolic thioredoxin system facilitates the import of mitochondrial small Tim proteins

(2012) Romina Durigon et al.   EMBO REPORTS

Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease

(2012) Eleonora Napoli et al.   HUMAN MOLECULAR GENETICS

Human CHCHD4 mitochondrial proteins regulate cellular oxygen consumption rate and metabolism and provide a critical role in hypoxia signaling and tumor progression

(2012) Jun Yang et al.   JOURNAL OF CLINICAL INVESTIGATION

Structural characterization of CHCHD5 and CHCHD7: Two atypical human twin CX9C proteins

(2012) Lucia Banci et al.   JOURNAL OF STRUCTURAL BIOLOGY

An Electron-Transfer Path through an Extended Disulfide Relay System: The Case of the Redox Protein ALR

(2012) Lucia Banci et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

In vivo evidence for cooperation of Mia40 and Erv1 in the oxidation of mitochondrial proteins

(2012) Lena Böttinger et al.   MOLECULAR BIOLOGY OF THE CELL

Disulfide Bond Formation: Sulfhydryl Oxidase ALR Controls Mitochondrial Biogenesis of Human MIA40

(2012) Malgorzata E. Sztolsztener et al.   TRAFFIC

Flavin-Linked Erv-Family Sulfhydryl Oxidases Release Superoxide Anion during Catalytic Turnover

(2011) Vidyadhar N. Daithankar et al.   BIOCHEMISTRY

Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis

(2011) Karina von der Malsburg et al.   DEVELOPMENTAL CELL

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

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

Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR

(2011) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial Disulfide Bond Formation Is Driven by Intersubunit Electron Transfer in Erv1 and Proofread by Glutathione

(2010) Melanie Bien et al.   MOLECULAR CELL

Molecular chaperone function of Mia40 triggers consecutive induced folding steps of the substrate in mitochondrial protein import

(2010) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Life with or without AIF

(2010) Emilie Hangen et al.   TRENDS IN BIOCHEMICAL SCIENCES

The Mitochondrial Disulfide Relay System Protein GFER Is Mutated in Autosomal-Recessive Myopathy with Cataract and Combined Respiratory-Chain Deficiency

(2009) Alessio Di Fonzo et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Targeting the hypoxia-inducible factor (HIF) pathway in cancer

(2009) Evon Poon et al.   EXPERT REVIEWS IN MOLECULAR MEDICINE

A novel intermembrane space–targeting signal docks cysteines onto Mia40 during mitochondrial oxidative folding

(2009) Dionisia P. Sideris et al.   JOURNAL OF CELL BIOLOGY

Redox-Linked Conformational Dynamics in Apoptosis-Inducing Factor

(2009) Irina F. Sevrioukova   JOURNAL OF MOLECULAR BIOLOGY

Identification of the Signal Directing Tim9 and Tim10 into the Intermembrane Space of Mitochondria

(2009) Dusanka Milenkovic et al.   MOLECULAR BIOLOGY OF THE CELL

MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria

(2009) Lucia Banci et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Structural basis of yeast Tim40/Mia40 as an oxidative translocator in the mitochondrial intermembrane space

(2009) S. Kawano et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural and Functional Roles of the Conserved Cysteine Residues of the Redox-regulated Import Receptor Mia40 in the Intermembrane Space of Mitochondria

(2008) Nadia Terziyska et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Biogenesis, Switching the Sorting Pathway of the Intermembrane Space Receptor Mia40

(2008) Agnieszka Chacinska et al.   JOURNAL OF BIOLOGICAL CHEMISTRY