M. Rugolo

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pH difference across the outer mitochondrial membrane measured with a green fluorescent protein mutant.

P. Pinton, R. Rizzuto, A. Porcelli, 2005, Biochemical and biophysical research communications.

Fine-tuning of the respiratory complexes stability and supercomplexes assembly in cells defective of complex III.

J. Fiori, F. Daldal, A. Ghelli, 2019, Biochimica et biophysica acta. Bioenergetics.

Metabolomics hallmarks OPA1 variants correlating with their in vitro phenotype and predicting clinical severity

D. Bonneau, G. Lenaers, P. Reynier, 2020, Human molecular genetics.

Respiratory Complex I Dysfunction Due to Mitochondrial DNA Mutations Shifts the Voltage Threshold for Opening of the Permeability Transition Pore toward Resting Levels*

A. Martinuzzi, A. Porcelli, A. Ghelli, 2009, Journal of Biological Chemistry.

Chloride transport pathways in human keratinocytes.

M. De Luca, M. Rugolo, T. Mastrocola, 1991, Advances in experimental medicine and biology.

ATP and A1 adenosine receptor agonists mobilize intracellular calcium and activate K+ and Cl- currents in normal and cystic fibrosis airway epithelial cells.

M. Rugolo, D. Gruenert, A. Rasola, 1993, The Journal of biological chemistry.

Volume regulation in human fibroblasts: role of Ca2+ and 5-lipoxygenase products in the activation of the Cl− efflux

E. Hoffmann, M. Rugolo, I. H. Lambert, 1993, The Journal of Membrane Biology.

Mitochondrial Respiratory Supercomplexes in Physiology and Diseases

A. Ghelli, M. Rugolo, V. Tropeano, 2015 .

Monitoring of the mitochondrial and plasma membrane potentials in human fibroblasts by tetraphenylphosphonium ion distribution

M. Rugolo, G. Lenaz, 1987, Journal of bioenergetics and biomembranes.

OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusion.

R. Youle, A. Porcelli, A. Ghelli, 2008, Brain : a journal of neurology.

Gamma rays induce a p53-independent mitochondrial biogenesis that is counter-regulated by HIF1α

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013, Cell Death and Disease.

Defective oxidative phosphorylation in thyroid oncocytic carcinoma is associated with pathogenic mitochondrial DNA mutations affecting complexes I and III.

E. Bonora, A. Martinuzzi, G. Tallini, 2006, Cancer research.

Caspase-independent death of Leber’s hereditary optic neuropathy cybrids is driven by energetic failure and mediated by AIF and Endonuclease G

A. Martinuzzi, A. Porcelli, A. Ghelli, 2005, Apoptosis.

Bioenergetics shapes cellular death pathways in Leber's hereditary optic neuropathy: a model of mitochondrial neurodegeneration.

A. Martinuzzi, A. Ghelli, C. Zanna, 2004, Biochimica et biophysica acta.

Respiratory complex I is essential to induce a Warburg profile in mitochondria-defective tumor cells

M. Attimonelli, P. Lollini, V. Giorgio, 2013, Cancer & Metabolism.

Reactive Oxygen Species Produced by Mutated Mitochondrial Respiratory Chains of Entire Cells Monitored Using Modified Microelectrodes

F. Paolucci, A. Ghelli, M. Rugolo, 2019, ChemElectroChem.

Complex II phosphorylation is triggered by unbalanced redox homeostasis in cells lacking complex III.

J. Fiori, F. Daldal, A. Ghelli, 2018, Biochimica et biophysica acta. Bioenergetics.

Mitochondria: Biogenesis and mitophagy balance in segregation and clonal expansion of mitochondrial DNA mutations.

C. Zanna, M. Rugolo, V. Carelli, 2015, The international journal of biochemistry & cell biology.

The genetic and metabolic signature of oncocytic transformation implicates HIF1alpha destabilization.

M. Attimonelli, A. Melcarne, A. Martinuzzi, 2010, Human molecular genetics.

Apoptotic Cell Death of Cybrid Cells Bearing Leber's Hereditary Optic Neuropathy Mutations Is Caspase Independent

A. Martinuzzi, A. Porcelli, A. Ghelli, 2003, Annals of the New York Academy of Sciences.

Coenzyme Q biosynthesis inhibition induces HIF‐1α stabilization and metabolic switch toward glycolysis

C. Torri, N. Rizzardi, N. Calonghi, 2020, The FEBS journal.

Lack of major mitochondrial bioenergetic changes in cultured skin fibroblasts from aged individuals.

M. Rugolo, M. L. Genova, G. Lenaz, 1994, Biochemistry and molecular biology international.

HIV-1 nef expression inhibits the activity of a Ca2+-dependent K+ channel involved in the control of the resting potential in CEM lymphocytes.

A. Porcelli, M. Rugolo, B. Rossi, 1999, Journal of immunology.

OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution.

P. Chinnery, P. Yu-Wai-Man, G. Lenaers, 2011, Genome research.

Supplementary Figure 13

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013 .

Protection against oxidant-induced apoptosis by exogenous glutathione in Leber hereditary optic neuropathy cybrids.

A. Martinuzzi, A. Porcelli, A. Ghelli, 2008, Investigative ophthalmology & visual science.

Syndromic parkinsonism and dementia associated with OPA 1 missense mutations

M. Zeviani, G. Rizzo, P. Barboni, 2015, Annals of neurology.

Eight human OPA1 isoforms, long and short: What are they for?

C. Zanna, M. Rugolo, V. Carelli, 2018, Biochimica et biophysica acta. Bioenergetics.

OPA1 Isoforms in the Hierarchical Organization of Mitochondrial Functions.

Prashant Mishra, G. Lenaers, J. McCaffery, 2017, Cell reports.

Sphingosylphosphorylcholine and sphingosinew-1-phosphate mobilize cytosolic calcium through different mechanisms in human airway epithelial cells

A. Porcelli, M. Rugolo, S. Hrelia, 1998 .

Glucose transport activation in human hematopoietic cells M07e is modulated by cytosolic calcium and calmodulin.

M. Rugolo, L. Landi, T. Maraldi, 2006, Cell calcium.

An inherited mitochondrial DNA disruptive mutation shifts to homoplasmy in oncocytic tumor cells

E. Bonora, G. Pasquinelli, A. Porcelli, 2009, Human mutation.

DNA Mutations Affecting Complexes I and III Carcinoma Is Associated with Pathogenic Mitochondrial Defective Oxidative Phosphorylation in Thyroid Oncocytic

E. Bonora, A. Martinuzzi, G. Tallini, 2006 .

The mechanism of action of beta-bungarotoxin at the presynaptic plasma membrane.

D. Nicholls, M. Rugolo, J. Dolly, 1986, The Biochemical journal.

7-Ketocholesterol and staurosporine induce opposite changes in intracellular pH, associated with distinct types of cell death in ECV304 cells.

A. Porcelli, A. Ghelli, C. Zanna, 2002, Archives of biochemistry and biophysics.

The effects of idebenone on mitochondrial bioenergetics

V. Giorgio, A. Ghelli, M. Rugolo, 2012, Biochimica et biophysica acta.

Supplementary Figure 10

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013 .

Intracellular pH regulation in U-2 OS human osteosarcoma cells transfected with P-glycoprotein.

N. Baldini, K. Scotlandi, A. Porcelli, 2002, Biochimica et biophysica acta.

Sphingosine-1-phosphate activates phospholipase D in human airway epithelial cells via a G protein-coupled receptor.

S. Spiegel, A. Porcelli, M. Rugolo, 2000, Archives of biochemistry and biophysics.

The antioxidant function of Bcl-2 preserves cytoskeletal stability of cells with defective respiratory complex I

A. Porcelli, A. Ghelli, C. Zanna, 2008, Cellular and Molecular Life Sciences.

Phosphate transport in mitochondria and submitochondrial particles: The influence of thiol oxidation

F. Zoccarato, M. Rugolo, D. Siliprandi, 1977, Journal of bioenergetics and biomembranes.

Magnesium Transport by Brain Mitochondria: Energy Requirement and Dependence on Ca2+ Fluxes

F. Zoccarato, M. Rugolo, 1984, Journal of neurochemistry.

Studies on the transport of carnitine in the brain using synaptosomes isolated from guinea-pig cerebral cortex.

F. Zoccarato, M. Rugolo, N. Siliprandi, 1983, Biochimica et biophysica acta.

F. Zoccarato, M. Rugolo, N. Siliprandi, 1981, European journal of biochemistry.

Involvement of endogenous phospholipase A2 in Ca2+ and Mg2+ movement induced by inorganic phosphate and diamide in rat liver mitochondria.

F. Zoccarato, M. Rugolo, A. Toninello, 1979, Biochemical and biophysical research communications.

'Behr syndrome' with OPA1 compound heterozygote mutations.

E. Bertini, R. Carrozzo, P. Barboni, 2015, Brain : a journal of neurology.

Efflux of magnesium and potassium ions from liver mitochondria induced by inorganic phosphate and by diamide

F. Zoccarato, M. Rugolo, A. Toninello, 1978, Journal of bioenergetics and biomembranes.

Hypotonic shock activated Cl- and K+ pathways in human fibroblasts.

M. Rugolo, T. Mastrocola, A. Flamigni, 1991, Biochimica et biophysica acta.

Chloride transport in human fibroblasts is activated by hypotonic shock.

M. Rugolo, G. Lenaz, A. Flamigni, 1989, Biochemical and biophysical research communications.

Changes in membrane permeability in cells infected by human cytomegalovirus

M. Rugolo, M. Landini, B. Baldassarri, 2005, Archives of Virology.

Oxidation of External NAD(P)H by Jerusalem Artichoke (Helianthus tuberosus) Mitochondria : A Kinetic and Inhibitor Study.

M. Rugolo, D. Zannoni, 1992, Plant physiology.

Characterization of chloride transport pathways in cultured human keratinocytes.

M. De Luca, M. Rugolo, T. Mastrocola, 1991, Biochimica et biophysica acta.

Activation of Ca2+-dependent K+ and Cl− currents by UTP and ATP in CFPAC-1 cells

M. Rugolo, T. Mastrocola, G. Romeo, 1994, Pflügers Archiv.

The phorbol ester PMA and cyclic AMP activate different Cl(-) and HCO3(-) fluxes in C127 cells expressing CFTR.

A. Porcelli, M. Rugolo, O. Zegarra‐Moran, 2001, Biochimica et biophysica acta.

Effects ofPolyamines on theOxidation ofExogenous NADHbyJerusalem Artichoke (Helianthus tuberosus) Mitochondrial

M. Rugolo, F. Antognoni, A. Flamigni, 1991 .

Effects of Polyamines on the Oxidation of Exogenous NADH by Jerusalem Artichoke (Helianthus tuberosus) Mitochondria.

M. Rugolo, D. Zannoni, F. Antognoni, 1991, Plant physiology.

Calcium ion transport in higher plant mitochondria (Helianthus tuberosus)

M. Rugolo, D. Zannoni, R. Pistocchi, 1990 .

Inhibition of the mitochondrial bc 1 complex by dibromothymoquinone

M. Rugolo, G. Lenaz, M. Esposti, 1983, FEBS letters.

Cellular and mitochondrial determination of low molecular mass organic acids by LC-MS/MS.

R. Gotti, J. Fiori, M. Rugolo, 2018, Journal of pharmaceutical and biomedical analysis.

Drug repositioning as a therapeutic strategy for neurodegenerations associated with OPA1 mutations

A. Ghelli, C. Zanna, M. Rugolo, 2020, Human molecular genetics.

Deciphering OPA1 mutations pathogenicity by combined analysis of human, mouse and yeast cell models.

C. La Morgia, F. Musiani, C. Zanna, 2018, Biochimica et biophysica acta. Molecular basis of disease.

OPA1: How much do we know to approach therapy?

G. Lenaers, C. Zanna, M. Rugolo, 2018, Pharmacological research.

Why mitochondria must fuse to maintain their genome integrity.

G. Lenaers, C. Zanna, M. Rugolo, 2013, Antioxidants & redox signaling.

A clinically complex form of dominant optic atrophy (OPA8) maps on chromosome 16.

P. Barboni, R. Lodi, R. Youle, 2011, Human molecular genetics.

OPA1 mutations induce mitochondrial DNA instability and optic atrophy 'plus' phenotypes.

R. Schwarzenbacher, D. Figarella-Branger, C. La Morgia, 2008, Brain : a journal of neurology.

Parallel efflux of Ca2+ and Pi in energized rat liver mitochondria.

M. Rugolo, A. Toninello, N. Siliprandi, 1981, The Biochemical journal.

Organization of the Respiratory Supercomplexes in Cells with Defective Complex III: Structural Features and Metabolic Consequences

A. Ghelli, C. Zanna, M. Rugolo, 2021, Life.

Kinetic alterations of cytochrome‐c oxidase in cystic fibrosis

M. Rugolo, G. Lenaz, G. Romeo, 1986, FEBS letters.

Disruptive mitochondrial DNA mutations in complex I subunits are markers of oncocytic phenotype in thyroid tumors

Giovanni Romeo, Giuseppe Gasparre, Anna Ghelli, 2007, Proceedings of the National Academy of Sciences.

Tetraphenylphosphonium Ion Distribution in Human Fibroblasts as a Function of In Vitro Aging

M. Rugolo, G. Lenaz, T. Mastrocola, 1988 .

Synergic action of calcium ions and diamide on mitochondrial swelling.

F. Zoccarato, M. Rugolo, A. Toninello, 1975, Biochemical and biophysical research communications.

Different mtDNA mutations modify tumor progression in dependence of the degree of respiratory complex I impairment.

P. Lollini, V. Giorgio, A. Porcelli, 2014, Human molecular genetics.

Respiratory complex I is essential to induce a Warburg profile in mitochondria-defective tumor cells

M. Attimonelli, P. Lollini, V. Giorgio, 2013, Cancer & metabolism.

A mutation threshold distinguishes the antitumorigenic effects of the mitochondrial gene MTND1, an oncojanus function.

Giovanni Romeo, Giuseppe Gasparre, Anna Ghelli, 2011, Cancer research.

the Control of the Resting Potential in CEM a Ca2+-Dependent K+ Channel Involved in HIV-1 Nef Expression Inhibits the Activity of

M. Porcelli, M. Rugolo, B. Rossi, 2013 .

Supplementary Figure 15

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013 .

Supplementary Figure 12

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013 .

Learning from oncocytic tumors: Why choose inefficient mitochondria?

A. Porcelli, M. Rugolo, G. Romeo, 2011, Biochimica et biophysica acta.

Molecular mechanisms of superoxide production by complex III: a bacterial versus human mitochondrial comparative case study.

F. Daldal, A. Ghelli, M. Rugolo, 2013, Biochimica et biophysica acta.

Increased chloride efflux in fibroblasts from X‐linked muscular dystrophies and clones from Duchenne carriers

M. Rocchi, M. Rugolo, G. Romeo, 1987, FEBS letters.

Phospholipase D stimulation is required for sphingosine-1-phosphate activation of actin stress fibre assembly in human airway epithelial cells.

A. Porcelli, A. Ghelli, M. Rugolo, 2002, Cellular signalling.

Extracellular 2‐chloroadenosine and ATP stimulate volume‐sensitive Cl− current and calcium mobilization in human tracheal 9HTEo− cells

M. Rugolo, D. Gruenert, M. Zottini, 1992, FEBS letters.

Leber's Hereditary Optic Neuropathy (LHON) Pathogenic Mutations Induce Mitochondrial-dependent Apoptotic Death in Transmitochondrial Cells Incubated with Galactose Medium*

A. Schapira, A. Martinuzzi, A. Porcelli, 2003, The Journal of Biological Chemistry.

The Background of Mitochondrial DNA Haplogroup J Increases the Sensitivity of Leber's Hereditary Optic Neuropathy Cells to 2,5-Hexanedione Toxicity

Anna Ghelli, Stefano Mattioli, Valerio Carelli, 2009, PloS one.

Rare mtDNA variants in Leber hereditary optic neuropathy families with recurrence of myoclonus

A. Torroni, P. Barboni, R. Lodi, 2008, Neurology.

The cytochrome b p.278Y>C mutation causative of a multisystem disorder enhances superoxide production and alters supramolecular interactions of respiratory chain complexes.

F. Daldal, A. Martinuzzi, J. Vissing, 2013, Human molecular genetics.

A Novel in‐Frame 18‐bp Microdeletion in MT‐CYB Causes a Multisystem Disorder with Prominent Exercise Intolerance

G. Rizzo, A. Torroni, P. Barboni, 2014, Human mutation.

Respiratory Complex I Dysfunction Due to Mitochondrial DNA Mutations Shifts the Voltage Threshold for Opening of the Permeability Transition Pore toward Resting Levels*

A. Martinuzzi, A. Porcelli, A. Ghelli, 2009, Journal of Biological Chemistry.

Increased accumulation of a lipophilic cation (tetraphenylphosphonium) in human embryo fibroblasts after infection with cytomegalovirus.

M. Rugolo, M. Landini, 1984, The Journal of general virology.

A volume-sensitive chloride conductance revealed in cultured human keratinocytes by 36Cl- efflux and whole-cell patch clamp recording.

M. De Luca, M. Rugolo, T. Mastrocola, 1992, Biochimica et biophysica acta.

alpha-latrotoxin of black widow spider venom depolarizes the plasma membrane, induces massive calcium influx, and stimulates transmitter release in guinea pig brain synaptosomes.

I. G. Scott, J. Meldolesi, D. Nicholls, 1982, Proceedings of the National Academy of Sciences of the United States of America.

Targeting of reporter molecules to mitochondria to measure calcium, ATP, and pH.

G. Rutter, P. Pinton, R. Rizzuto, 2001, Methods in cell biology.

Clonal expansion of mtDNA deletions: different disease models assessed by digital droplet PCR in single muscle cells

P. Yu-Wai-Man, R. Horvath, M. Rugolo, 2018, Scientific Reports.

ATP depletion and caspase independent death of cybrids bearing Leber's Hereditary Optic neuropathy MTDNA mutations incubated in galactose medium. A model for endonuclease G-mediated apoptosis

A. Porcelli, A. Ghelli, C. Zanna, 2004 .

Pertussis toxin- and PMA-insensitive calcium mobilization by sphingosine in CFPAC-1 cells: evidence for a phosphatidic acid-dependent mechanism.

M. Rugolo, S. Hrelia, S. Orlati, 1997, Biochimica et biophysica acta.

Sphingosine-induced inhibition of capacitative calcium influx in CFPAC-1 cells.

M. Rugolo, M. Cavazzoni, S. Orlati, 1996, Cell calcium.

Apoptosis induced by staurosporine in ECV304 cells requires cell shrinkage and upregulation of Cl− conductance

S. Ferroni, A. Porcelli, A. Ghelli, 2004, Cell Death and Differentiation.

Arachidonic acid release by ionomycin and phorbol ester is similar in C127 epithelial cells expressing wild-type or mutated (ΔF508) cystic fibrosis transmembrane conductance regulator

A. Porcelli, A. Ghelli, M. Rugolo, 1999, Cellular and Molecular Life Sciences CMLS.

The response of chloride transport to cyclic AMP, calcium and hypotonic shock in normal and cystic fibrosis fibroblasts.

M. Rugolo, T. Mastrocola, 1990, Life sciences.

Kinetic analysis of chloride efflux from normal and cystic fibrosis fibroblasts.

M. Rugolo, G. Romeo, G. Lenaz, 1986, Biochemical and biophysical research communications.

Supplementary Figure 9

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013 .

Alterations in the supramolecular interactions of respiratory chain complexes and enhanced superoxide production by the cytochrome b Y278C mutation which causes a multisystem disorder

F. Daldal, A. Martinuzzi, J. Vissing, 2012 .

Histamine activates phospholipase C in human airway epithelial cells via a phorbol ester-sensitive pathway.

M. Rugolo, D. Gruenert, S. Hrelia, 1996, The American journal of physiology.

The response of chloride transport to cyclic AMP, calcium and hypotonic shock in normal and cystic fibrosis fibroblasts.

M. Rugolo, T. Mastrocola, 1991, Advances in experimental medicine and biology.

Role of CFTR and anion exchanger in bicarbonate fluxes in C127 cell lines

A. Porcelli, M. Rugolo, T. Mastrocola, 1998, FEBS letters.

Supplementary Figure 14

L. Eusebi, L. Fuccio, M. F. Caratozzolo, 2013 .

A mutation threshold distinguishes the antitumorigenic effects of the mitochondrial gene MTND1, an oncojanus function.

P. Lollini, Christine Betts, K. Scotlandi, 2011, Cancer research.

Oxygen-induced inhibition of light-dependent uptake of tetraphenylphosphonium ions as a probe of a direct interaction between photosynthetic and respiratory components in cells of Rhodopseudomonas capsulata.

M. Rugolo, D. Zannoni, 1983, Biochemical and biophysical research communications.

Different mtDNA mutations modify tumor progression in dependence of the degree of respiratory complex I impairment.

P. Lollini, V. Giorgio, A. Porcelli, 2014, Human molecular genetics.

The genetic and metabolic signature of oncocytic transformation implicates HIF1alpha destabilization.

M. Attimonelli, A. Melcarne, A. Martinuzzi, 2010, Human molecular genetics.

Why mitochondria must fuse to maintain their genome integrity.

G. Lenaers, C. Zanna, M. Rugolo, 2013, Antioxidants & redox signaling.