C. Moraes

发表

Reactive oxygen species and mitochondrial diseases.

I. Kirkinezos, C. Moraes, Ilias G. Kirkinezos, 2001, Seminars in cell & developmental biology.

PGC-1α/β upregulation is associated with improved oxidative phosphorylation in cells harboring nonsense mtDNA mutations

S. Srivastava, C. Moraes, J. Barrett, 2007 .

Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype.

B. Spiegelman, C. Moraes, F. Diaz, 2008, Cell metabolism.

Fast adaptive coevolution of nuclear and mitochondrial subunits of ATP synthetase in orangutan.

S. Müller, M. Bayona-Bafaluy, C. Moraes, 2005, Molecular biology and evolution.

Role of Cytochrome c in Apoptosis: Increased Sensitivity to Tumor Necrosis Factor Alpha Is Associated with Respiratory Defects but Not with Lack of Cytochrome c Release

J. Millán, L. Boise, C. Moraes, 2007, Molecular and Cellular Biology.

Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.

A. Torraco, C. Moraes, S. Garcia, 2014, Human molecular genetics.

Generation of histocompatible tissues using nuclear transplantation

James J. Yoo, S. Soker, A. Atala, 2002, Nature Biotechnology.

Increased mitochondrial biogenesis in muscle improves aging phenotypes in the mtDNA mutator mouse.

T. Prolla, C. Moraes, J. Lincoln, 2012, Human molecular genetics.

Clinical and morphologic features of a myopathy associated with a point mutation in the mitochondrial tRNAPro gene

S. Dimauro, M. Hart, C. Moraes, 1994, Neurology.

Extremely high levels of mutant mtDNAs co-localize with cytochrome c oxidase-negative ragged-red fibers in patients harboring a point mutation at nt 3243.

S. Dimauro, E. Schon, M. Sano, 1994, Human molecular genetics.

Segregation patterns of a novel mutation in the mitochondrial tRNA glutamic acid gene associated with myopathy and diabetes mellitus.

S. Dimauro, C. Moraes, E. Bonilla, 1995, American journal of human genetics.

A MERRF/PEO overlap syndrome associated with the mitochondria1 DNA 3243 mutation

W. Bradley, C. Moraes, A. Verma, 1996, Neurology.

A novel myopathy-associated mitochondrial DNA mutation altering the conserved size of the tRNAGln anticodon loop

C. Moraes, C. Tengan, R. Dey, 2000, Neuromuscular Disorders.

MitoTALEN reduces mutant mtDNA load and restores tRNAAla levels in a mouse model of heteroplasmic mtDNA mutation

C. Moraes, N. Larsson, J. Stewart, 2018, Nature Medicine.

Oxidative phosphorylation dysfunction does not increase the rate of accumulation of age-related mtDNA deletions in skeletal muscle.

M. Zeviani, C. Moraes, S. Shanske, 1997, Mutation research.

Depletion of muscle mitochondrial DNA in AIDS patients with zidovudine-induced myopathy

S. Dimauro, E. Schon, M. Dalakas, 1991, The Lancet.

RETRACTED: Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy.

C. Moraes, F. Diaz, T. Wenz, 2013, Mitochondrion.

Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency

M. Baumgartner, D. di Bernardo, H. Debaix, 2020, Nature Communications.

Modulating mtDNA heteroplasmy by mitochondria-targeted restriction endonucleases in a ‘differential multiple cleavage-site’ model

C. Moraes, S. Bacman, S. Williams, 2007, Gene Therapy.

Photobiomodulation enhancement of cell proliferation at 660 nm does not require cytochrome c oxidase.

C. Moraes, F. Diaz, G. Gavini, 2019, Journal of photochemistry and photobiology. B, Biology.

ATAD3 controls mitochondrial cristae structure in mouse muscle, influencing mtDNA replication and cholesterol levels

C. Moraes, S. Garcia, F. Diaz, 2018, Journal of Cell Science.

Mitochondrial encephalomyopathies.

S. Dimauro, E. Schon, M. Zeviani, 1989, Progress in clinical and biological research.

The CoQH2/CoQ Ratio Serves as a Sensor of Respiratory Chain Efficiency.

P. Navas, J. Enríquez, E. Calvo, 2016, Cell reports.

Expanding the functional human mitochondrial DNA database by the establishment of primate xenomitochondrial cybrids.

C. Moraes, L. Kenyon, 1997, Proceedings of the National Academy of Sciences of the United States of America.

Mild deficiency of mitochondrial Complex III in a mouse model of Alzheimer’s disease decreases amyloid beta plaque formation

C. Moraes, F. Diaz, R. Brambilla, 2021 .

The mitochondrial DNA polymerase gamma degrades linear DNA fragments precluding the formation of deletions

C. Moraes, Melanie J. Plastini, S. Bacman, 2018, Nature Communications.

Lack of Parkin Anticipates the Phenotype and Affects Mitochondrial Morphology and mtDNA Levels in a Mouse Model of Parkinson's Disease

C. Moraes, Nadee Nissanka, M. Pinto, 2017, The Journal of Neuroscience.

Image-Based Analysis of Mitochondrial Area and Counting from Adult Mouse Dopaminergic Neurites.

C. Moraes, Nadee Nissanka, M. Pinto, 2018, Bio-protocol.

Cellular adaptations to oxidative phosphorylation defects in cancer

S. Srivastava, C. Moraes, 2009 .

Pioglitazone ameliorates the phenotype of a novel Parkinson’s disease mouse model by reducing neuroinflammation

C. Moraes, F. Diaz, R. Brambilla, 2016, Molecular Neurodegeneration.

Titrating the Effects of Mitochondrial Complex I Impairment in the Cell Physiology*

C. Moraes, A. Barrientos, 1999, The Journal of Biological Chemistry.

Long-Term Bezafibrate Treatment Improves Skin and Spleen Phenotypes of the mtDNA Mutator Mouse

T. Prolla, C. Moraes, S. Garcia, 2012, PloS one.

A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations

C. Moraes, M. Marini, T. Wenz, 2011, Journal of cellular and molecular medicine.

Enhanced Transcriptional Activity andMitochondrial Localization of STAT 3 Co-induce Axon Regrowth in the Adult Central Nervous System Graphical Abstract Highlights

J. Bixby, K. Park, V. Lemmon, 2016 .

Enhanced Transcriptional Activity and Mitochondrial Localization of STAT3 Co-induce Axon Regrowth in the Adult Central Nervous System.

J. Bixby, K. Park, V. Lemmon, 2016, Cell reports.

Cytochrome c Association with the Inner Mitochondrial Membrane Is Impaired in the CNS of G93A-SOD1 Mice

W. Bradley, J. Oca-Cossio, M. Perez-Pinzon, 2005, The Journal of Neuroscience.

Aberrant δPKC activation in the spinal cord of Wobbler mouse: a model of motor neuron disease

W. Bradley, M. Perez-Pinzon, I. Kirkinezos, 2005, Neurobiology of Disease.

Dysfunctional mitochondrial respiration in the wobbler mouse brain

R. Busto, W. Bradley, T. Sick, 2001, Neuroscience Letters.

Double-strand breaks of mouse muscle mtDNA promote large deletions similar to multiple mtDNA deletions in humans.

S. Srivastava, C. Moraes, 2005, Human molecular genetics.

Increase in Muscle Mitochondrial Biogenesis Does Not Prevent Muscle Loss but Increased Tumor Size in a Mouse Model of Acute Cancer-Induced Cachexia

T. Zimmers, C. Moraes, Alicia M. Pickrell, 2012, PloS one.

Deletion of mitochondrial DNA in patients with combined features of kearns‐sayre and MELAS syndromes

S. Dimauro, C. Langman, M. Zupanc, 1991, Annals of neurology.

Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency

C. Moraes, S. Garcia, F. Diaz, 2020, JCI insight.

Endurance exercise is protective for mice with mitochondrial myopathy.

C. Moraes, F. Diaz, T. Wenz, 2009, Journal of applied physiology.

Myopathy reversion in mice after restauration of mitochondrial complex I

C. Moraes, F. Diaz, Susana Peralta, 2020, EMBO molecular medicine.

Specific elimination of mutant mitochondrial genomes in patient–derived cells by mitoTALENs

C. Moraes, Susana Peralta, S. Williams, 2013, Nature Medicine.

An ALS mouse model with a permeable blood–brain barrier benefits from systemic cyclosporine A treatment

W. Bradley, I. Kirkinezos, C. Moraes, 2004, Journal of neurochemistry.

Cells Lacking Rieske Iron-Sulfur Protein Have a Reactive Oxygen Species-Associated Decrease in Respiratory Complexes I and IV

J. Enríquez, C. Moraes, F. Diaz, 2011, Molecular and Cellular Biology.

Adult-Onset Deficiency of Mitochondrial Complex III in a Mouse Model of Alzheimer’s Disease Decreases Amyloid Beta Plaque Formation

C. Moraes, F. Diaz, R. Brambilla, 2022, Molecular Neurobiology.

Regulation of Respiration and Apoptosis by Cytochrome c Threonine 58 Phosphorylation

B. Edwards, L. Grossman, M. Hüttemann, 2019, Scientific Reports.

PGC-1alpha/beta induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders.

S. Srivastava, C. Moraes, F. Diaz, 2009, Human molecular genetics.

A helicase is born

C. Moraes, 2001, Nature Genetics.

The role of PGC‐1 coactivators in aging skeletal muscle and heart

A. Rebelo, C. Moraes, Lloye M. Dillon, 2012, IUBMB life.

PGC-1alpha/beta upregulation is associated with improved oxidative phosphorylation in cells harboring nonsense mtDNA mutations.

S. Srivastava, C. Moraes, J. Barrett, 2007, Human molecular genetics.

Mutations of cytochrome c identified in patients with thrombocytopenia THC4 affect both apoptosis and cellular bioenergetics.

A. Pastore, A. Savoia, L. Salviati, 2014, Biochimica et biophysica acta.

Precise and simultaneous quantification of mitochondrial DNA heteroplasmy and copy number by digital PCR

C. Moraes, S. Bacman, Wendy Shoop, 2022, The Journal of biological chemistry.

Two novel pathogenic mitochondrial DNA mutations affecting organelle number and protein synthesis. Is the tRNA(Leu(UUR)) gene an etiologic hot spot?

S. Dimauro, E. Schon, R. Lovelace, 1993, The Journal of clinical investigation.

Making the most of what you've got: Optimizing residual OXPHOS function in mitochondrial diseases

C. Moraes, 2009, EMBO molecular medicine.

Lack of Cytochrome c in Mouse Fibroblasts Disrupts Assembly/Stability of Respiratory Complexes I and IV*

Xianlin Han, C. Moraes, U. Vempati, 2009, Journal of Biological Chemistry.

DNA‐editing enzymes as potential treatments for heteroplasmic mtDNA diseases

C. Moraes, S. Bacman, U. Zekonyte, 2020, Journal of internal medicine.

Mitochondrial genome engineering coming-of-age.

C. Moraes, Jose Domingo Barrera-Paez, 2022, Trends in genetics : TIG.

The Role of Cytochrome c in Apoptosis: Increased Sensitivity to TNF-α is Associated with Respiratory Defects But Not with Lack of Cytochrome c Release.

J. Millán, L. Boise, C. Moraes, 2007 .

RAPID COMMUNICATION Immunocytochemical Study of Dystrophin in Muscle Culturesfrom Patients with Duchenne Muscular Dystrophy and Unaffected Control Patients

S. Dimauro, A. Hays, C. Moraes, 2022 .

Erythromycin as a potential precipitating agent in the onset of Leber's hereditary optic neuropathy.

B. Lam, C. Moraes, C. Luca, 2004, Mitochondrion.

The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis?

H. Fukui, C. Moraes, 2008, Trends in Neurosciences.

BCL-2 Improves Oxidative Phosphorylation and Modulates Adenine Nucleotide Translocation in Mitochondria of Cells Harboring Mutant mtDNA*

J. Oca-Cossio, M. Woischnik, C. Moraes, 2003, The Journal of Biological Chemistry.

Intra-and intermolecular recombination of mitochondrial DNA after in vivo induction of multiple double-strand breaks

C. Moraes, S. Williams, S. Bacman, 2009 .

Limitations of allotopic expression of mitochondrial genes in mammalian cells.

J. Oca-Cossio, C. Moraes, H. Hao, 2003, Genetics.

Detection and analysis of mitochondrial DNA and RNA in muscle by in situ hybridization and single-fiber PCR.

E. Schon, C. Moraes, 1996, Methods in enzymology.

Transmitochondrial technology in animal cells.

C. Moraes, S. Bacman, 2007, Methods in cell biology.

Adrenoleukodystrophy and the mitochondrial connection: clues for supplementing Lorenzo's oil.

C. Moraes, 2013, Brain : a journal of neurology.

Mitochondrial DNA depletion: Mutations in thymidine kinase gene with myopathy and SMA

S. Dimauro, M. Mancuso, A. Marina, 2002 .

Cre recombinase activity is inhibited in vivo but not ex vivo by a mutation in the asymmetric spacer region of the distal loxP site

C. Moraes, T. Arguello, 2015, Genesis.

cultured rat CNS neurons Mitochondrial dysfunction induced by heat stress in

C. Moraes, E. Barrett, D. Nonner, 2014 .

Mitochondrial dysfunction induced by heat stress in cultured rat CNS neurons.

C. Moraes, E. Barrett, J. Barrett, 2012, Journal of neurophysiology.

Glycolytic oligodendrocytes maintain myelin and long-term axonal integrity

Jens Frahm, Ueli Suter, Susann Boretius, 2012, Nature.

Selective Elimination of Mitochondrial Mutations in the Germline by Genome Editing

J. C. Belmonte, I. Sancho-Martinez, Keiichiro Suzuki, 2015, Cell.

Mitochondrial Genome Engineering: The Revolution May Not Be CRISPR-Ized

M. Minczuk, C. Moraes, P. Gammage, 2017, Trends in genetics : TIG.

Current strategies towards therapeutic manipulation of mtDNA heteroplasmy.

C. Moraes, C. Pereira, 2017, Frontiers in bioscience.

Manipulation of mtDNA heteroplasmy in all striated muscles of newborn mice by AAV9-mediated delivery of a mitochondria targeted restriction endonuclease

D. Duan, C. Moraes, S. Williams, 2011, Gene Therapy.

Organ-specific shifts in mtDNA heteroplasmy following systemic delivery of a mitochondria-targeted restriction endonuclease

C. Moraes, S. Garcia, S. Williams, 2010, Gene Therapy.

Rapid directional shift of mitochondrial DNA heteroplasmy in animal tissues by a mitochondrially targeted restriction endonuclease.

E. Shoubridge, M. Bayona-Bafaluy, C. Moraes, 2005, Proceedings of the National Academy of Sciences of the United States of America.

Cloning of an endangered species (Bos gaurus) using interspecies nuclear transfer.

M. West, R. Lanza, C. Moraes, 2000, Cloning.

Mitochondrial alterations during carcinogenesis: a review of metabolic transformation and targets for anticancer treatments.

C. Moraes, Susana Peralta, Xiao Wang, 2013, Advances in cancer research.

mtDNA depletion with variable tissue expression: a novel genetic abnormality in mitochondrial diseases.

S. Dimauro, E. Schon, H. Tritschler, 1991, American journal of human genetics.

Mitochondrial respiration controls neoangiogenesis during wound healing and tumour growth

M. Pasparakis, S. Brodesser, O. Coutelle, 2020, Nature Communications.

The role of cytochrome c oxidase deficiency in ROS and amyloid plaque formation

H. Fukui, C. Moraes, Alicia M. Pickrell, 2009, Journal of bioenergetics and biomembranes.

Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease

Hirokazu Fukui, H. Fukui, C. Moraes, 2007, Proceedings of the National Academy of Sciences.

Jcb: Article

J. Eisenbart, N. Chandel, M. Murphy, 2022 .

A 3' UTR modification of the mitochondrial rieske iron sulfur protein in mice produces a specific skin pigmentation phenotype.

C. Moraes, S. Garcia, F. Diaz, 2008, Journal of Investigative Dermatology.

A direct repeat is a hotspot for large-scale deletion of human mitochondrial DNA.

S. Dimauro, E. Schon, M. Zeviani, 1989, Science.

MELAS: Clinical features, biochemistry, and molecular genetics

S. Dimauro, E. Schon, A. Martinuzzi, 1992, Annals of neurology.

Functional Constraints of Nuclear-Mitochondrial DNA Interactions in Xenomitochondrial Rodent Cell Lines* 210

C. Moraes, A. Barrientos, R. Dey, 2000, The Journal of Biological Chemistry.

A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions.

K. Padgett, C. Moraes, S. Garcia, 2012, Human molecular genetics.

Mouse models of oxidative phosphorylation dysfunction and disease.

A. Torraco, C. Moraes, U. Vempati, 2008, Methods.

Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency.

Christine K. Thomas, C. Moraes, S. Garcia, 2005, Human molecular genetics.

Mitochondrial DNA structure and function.

J. Oca-Cossio, S. Srivastava, I. Kirkinezos, 2002, International review of neurobiology.

What regulates mitochondrial DNA copy number in animal cells?

C. Moraes, 2001, Trends in genetics : TIG.

Cybrid technology.

C. Moraes, S. Bacman, Nadee Nissanka, 2020, Methods in cell biology.

Transmitochondrial technology in animal cells.

C. Moraes, A. Barrientos, R. Dey, 2001, Methods in cell biology.

The Striatum Is Highly Susceptible to Mitochondrial Oxidative Phosphorylation Dysfunctions

H. Fukui, C. Moraes, Alicia M. Pickrell, 2011, The Journal of Neuroscience.

Mitochondrial DNA depletion

S. Dimauro, M. Mancuso, A. Marina, 2002, Neurology.

Mitochondrial myopathy of childhood associated with depletion of mitochondrial DNA

S. Dimauro, E. Schon, H. Tritschler, 1992, Neurology.

Mitochondrial transcription: lessons from mouse models.

C. Moraes, Susana Peralta, Xiao Wang, 2012, Biochimica et biophysica acta.

Deletions of mitochondrial DNA in Kearns-Sayre syndrome

S. Dimauro, E. Schon, M. Zeviani, 1998, Neurology.

Deletions of mitochondrial DNA in Kearns‐Sayre syndrome

S. Dimauro, E. Schon, M. Zeviani, 1988, Neurology.

Respiratory supercomplexes act as a platform for complex III‐mediated maturation of human mitochondrial complexes I and IV

Massimo Zeviani, Cristina Ugalde, M. Zeviani, 2020, The EMBO journal.

Cytochrome oxidase deficiency: progress and problems

S. Dimauro, E. Schon, C. Moraes, 1994 .

Mitochondrial encephalomyopathies: biochemical approach.

S. Dimauro, E. Schon, H. Tritschler, 1991, Revue neurologique.

Mitochondrial myopathy induces a starvation-like response.

K. Pietiläinen, M. Orešič, I. Hovatta, 2010, Human molecular genetics.

Mitochondrial genome changes and neurodegenerative diseases.

C. Moraes, M. Pinto, 2014, Biochimica et biophysica acta.

A chemical enucleation method for the transfer of mitochondrial DNA to ρ° cells

M. Bayona-Bafaluy, C. Moraes, G. Manfredi, 2003 .

A chemical enucleation method for the transfer of mitochondrial DNA to rho(o) cells.

M. Bayona-Bafaluy, C. Moraes, G. Manfredi, 2003, Nucleic acids research.

Mitochondrial DNA damage in a mouse model of Alzheimer's disease decreases amyloid beta plaque formation

H. Fukui, C. Moraes, Alicia M. Pickrell, 2013, Neurobiology of Aging.

Cybrids in Alzheimer's disease: A cellular model of the disease?

E. Shoubridge, E. Schon, C. Moraes, 1998, Neurology.

Regular exercise is beneficial to a mouse model of amyotrophic lateral sclerosis

W. Bradley, C. Moraes, D. Hernandez, 2003, Annals of neurology.

NO control of mitochondrial function in normal and transformed cells.

C. Moraes, C. Tengan, 2017, Biochimica et biophysica acta. Bioenergetics.

Mitochondrial DNA damage and reactive oxygen species in neurodegenerative disease

C. Moraes, Nadee Nissanka, 2018, FEBS letters.

Analysis of giant deletions of human mitochondrial DNA in progressive external ophthalmoplegia

S. Dimauro, E. Schon, M. Zeviani, 1991 .

Mitochondrial biogenesis and turnover.

C. Moraes, F. Diaz, 2008, Cell calcium.

A new mutation associated with MELAS is located in a mitochondrial DNA polypeptide-coding gene

S. Dimauro, E. Schon, C. Moraes, 1995, Neuromuscular Disorders.

Targeted Mitochondrial Genome Elimination

C. Moraes, S. Bacman, C. Pereira, 2018 .

Lack of Oxidative Phosphorylation and Low Mitochondrial Membrane Potential Decrease Susceptibility to Apoptosis and Do Not Modulate the Protective Effect of Bcl-xL in Osteosarcoma Cells*

C. Moraes, Runu Dey, Carlos T. Moraes, 2000, The Journal of Biological Chemistry.

What role does mitochondrial stress play in neurodegenerative diseases?

C. Moraes, Alicia M. Pickrell, 2010, Methods in molecular biology.

In vivo methylation of mtDNA reveals the dynamics of protein–mtDNA interactions

A. Rebelo, Sion L. Williams, C. Moraes, 2009, Nucleic acids research.

Human mitochondrial DNA with large deletions repopulates organelles faster than full-length genomes under relaxed copy number control.

M. Bayona-Bafaluy, C. Moraes, F. Diaz, 2002, Nucleic acids research.

Transient systemic mtDNA damage leads to muscle wasting by reducing the satellite cell pool.

C. Moraes, Alicia M. Pickrell, Lloye M. Dillon, 2013, Human molecular genetics.

Transcriptional co-expression and co-regulation of genes coding for components of the oxidative phosphorylation system

C. Moraes, Carlos T Moraes, Corina van Waveren, 2008, BMC Genomics.

The mitochondrial tRNA(Leu(UUR)) mutation in mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS): genetic, biochemical, and morphological correlations in skeletal muscle.

S. Dimauro, E. Schon, E. Ricci, 1992, American journal of human genetics.

Clinical and Biochemical Studies on Cytochrome Oxidase Deficiencies a

S. Dimauro, E. Schon, M. Zeviani, 1988, Annals of the New York Academy of Sciences.

Somatic mtDNA Mutation Spectra in the Aging Human Putamen

Deborah C. Mash, Stephan Züchner, D. Mash, 2013, PLoS genetics.

Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species

Sion L. Williams, T. Boettger, T. Braun, 2013, Proceedings of the National Academy of Sciences.

The mtDNA mutation spectrum of the progeroid Polg mutator mouse includes abundant control region multimers.

T. Prolla, S. Züchner, J. Vance, 2010, Cell metabolism.

Intra- and inter-molecular recombination of mitochondrial DNA after in vivo induction of multiple double-strand breaks

C. Moraes, S. Williams, S. Bacman, 2009, Nucleic acids research.

Transient mitochondrial DNA double strand breaks in mice cause accelerated aging phenotypes in a ROS-dependent but p53/p21-independent manner

Paul D. Morton, T. Malek, C. Moraes, 2016, Cell Death and Differentiation.

Cytochrome c oxidase assembly in primates is sensitive to small evolutionary variations in amino acid sequence.

J. Wienberg, S. Müller, C. Moraes, 2000, Molecular biology and evolution.

Molecular analysis of the muscle pathology associated with mitochondrial DNA deletions

S. Dimauro, E. Schon, V. Petruzzella, 1992, Nature Genetics.

Simultaneous transfer of mitochondrial DNA and single chromosomes in somatic cells: a novel approach for the study of defects in nuclear-mitochondrial communication.

C. Moraes, A. Barrientos, 1998, Human molecular genetics.

Pattern of organization of human mitochondrial pseudogenes in the nuclear genome.

M. Woischnik, C. Moraes, 2002, Genome research.

Human Xenomitochondrial Cybrids

C. Moraes, A. Barrientos, L. Kenyon, 1998, The Journal of Biological Chemistry.

Increased muscle PGC-1α expression protects from sarcopenia and metabolic disease during aging

B. Spiegelman, C. Moraes, T. Wenz, 2009, Proceedings of the National Academy of Sciences.

Oxidative phosphorylation dysfunction modulates expression of extracellular matrix--remodeling genes and invasion.

Yubo Sun, H. Cheung, C. Moraes, 2006, Carcinogenesis.

Loss of Mcl-1 Protein and Inhibition of Electron Transport Chain Together Induce Anoxic Cell Death

A. Strasser, Emelyn H. Shroff, R. Flavell, 2006, Molecular and Cellular Biology.

Nuclear-Mitochondrial Interactions

C. Moraes, B. Walker, 2022, Biomolecules.

ATAD3A has a scaffolding role regulating mitochondria inner membrane structure and protein assembly

H. Antonicka, R. Shiekhattar, G. Gaidosh, 2021, Cell reports.

Widespread tissue distribution of mitochondrial DNA deletions in Kearns‐Sayre syndrome

S. Dimauro, C. Moraes, A. Lombès, 1990, Neurology.

Increases in mitochondrial biogenesis impair carcinogenesis at multiple levels

C. Moraes, Xiao Wang, 2011, Molecular oncology.

Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome.

S. Dimauro, E. Schon, M. Zeviani, 1989, The New England journal of medicine.

Mitochondrial DNA transcription regulation and nucleoid organization

A. Rebelo, C. Moraes, Lloye M. Dillon, 2011, Journal of Inherited Metabolic Disease.

Mouse models of oxidative phosphorylation defects: powerful tools to study the pathobiology of mitochondrial diseases.

A. Torraco, C. Moraes, F. Diaz, 2009, Biochimica et biophysica acta.

A new mtDNA mutation in the tRNA(Lys) gene associated with myoclonic epilepsy and ragged-red fibers (MERRF).

S. Dimauro, G. Silvestri, S. Oh, 1992, American journal of human genetics.

Mitochondrial methionyl N-formylation affects steady-state levels of oxidative phosphorylation complexes and their organization into supercomplexes

C. Moraes, U. RajBhandary, T. Arguello, 2018, The Journal of Biological Chemistry.

Techniques and pitfalls in the detection of pathogenic mitochondrial DNA mutations.

D. Atencio, J. Oca-Cossio, C. Moraes, 2003, The Journal of molecular diagnostics : JMD.

Decreased mitochondrial tRNALys steady-state levels and aminoacylation are associated with the pathogenic G8313A mitochondrial DNA mutation.

D. Atencio, C. Moraes, S. Bacman, 2003, The Biochemical journal.

Characteristics of Mitochondrial DNA Diseases

C. Moraes, 1998 .

A Novel Mitochondrial G8313A Mutation Associated with Prominent Initial Gastrointestinal Symptoms and Progressive Encephaloneuropathy

S. Dimauro, G. Berry, C. Moraes, 1997, Pediatric Research.

Identification of a mutation in the mitochondrial tRNACys gene associated with mitochondrial encephalopathy

S. Dimauro, E. Schon, C. Moraes, 1996, Human mutation.

A mitochondrial tRNA anticodon swap associated with a muscle disease

S. Dimauro, E. Schon, C. Moraes, 1993, Nature Genetics.

SCO2 mutations cause early-onset axonal Charcot-Marie-Tooth disease associated with cellular copper deficiency

E. Schon, K. Mathews, A. Rebelo, 2018, Brain : a journal of neurology.

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

T. Wai, P. Cobine, A. Hossain, 2015, Cell reports.

Mitochondrial function in heart muscle from patients with idiopathic dilated cardiomyopathy.

Ò. Miró, C. Moraes, A. Barrientos, 2000, Cardiovascular research.

Microdissection and analytical PCR for the investigation of mtDNA lesions.

Sion L. Williams, C. Moraes, 2007, Methods in cell biology.

Respiratory complex III is required to maintain complex I in mammalian mitochondria.

C. Bruno, J. Enríquez, M. Bayona-Bafaluy, 2004, Molecular cell.

Mitochondrial encephalomyopathy and hypoparathyroidism associated with a duplication and a deletion of mitochondrial deoxyribonucleic acid.

C. Moraes, M. Rocha, A. Gabbai, 1998, The Journal of clinical endocrinology and metabolism.

Structural and functional mitochondrial abnormalities associated with high levels of partially deleted mitochondrial DNAs in somatic cell hybrids

C. Moraes, K. Tanji, A. Miranda, 1992, Somatic cell and molecular genetics.

Recombination via flanking direct repeats is a major cause of large-scale deletions of human mitochondrial DNA.

S. Dimauro, E. Schon, R. Rizzuto, 1990, Nucleic acids research.

Mechanisms of human mitochondrial DNA maintenance: the determining role of primary sequence and length over function.

C. Moraes, H. Hao, L. Kenyon, 1999, Molecular biology of the cell.

Mechanisms of formation and accumulation of mitochondrial DNA deletions in aging neurons.

H. Fukui, C. Moraes, 2009, Human molecular genetics.

Mitochondrial DNA Base Editing: Good Editing Things Still Come in Small Packages.

C. Moraes, S. Bacman, 2020, Molecular cell.

ρ0 tumor cells: a model for studying whether mitochondria are targets for rhodamine 123, doxorubicin, and other drugs

N. Savaraj, T. Lampidis, C. Moraes, 2000 .

Mitochondrial DNA heteroplasmy in disease and targeted nuclease‐based therapeutic approaches

C. Moraes, Nadee Nissanka, 2020, EMBO reports.

Mechanisms of Mitochondrial DNA Deletion Formation.

M. Minczuk, C. Moraes, Nadee Nissanka, 2019, Trends in genetics : TIG.

Heteroplasmy of mitochondrial genomes in clonal cultures from patients with Kearns-Sayre syndrome.

S. Dimauro, E. Schon, C. Moraes, 1989, Biochemical and biophysical research communications.

Manipulation of mitochondrial genes and mtDNA heteroplasmy.

M. Minczuk, C. Moraes, P. Gammage, 2020, Methods in cell biology.

Emerging therapeutic approaches to mitochondrial diseases.

Sion L. Williams, C. Moraes, T. Wenz, 2010, Developmental disabilities research reviews.

Respiration-Deficient Astrocytes Survive As Glycolytic Cells In Vivo

M. Götz, K. Nave, S. Boretius, 2017, The Journal of Neuroscience.

Mechanisms linking mtDNA damage and aging.

C. Moraes, M. Pinto, 2013, Free radical biology & medicine.

Mitochondrial involvement in amyotrophic lateral sclerosis

W. Bradley, C. Moraes, S. Bacman, 2007, Molecular Neurobiology.

The mitochondrial DNA polymerase gamma degrades linear DNA fragments precluding the formation of deletions

C. Moraes, Melanie J. Plastini, S. Bacman, 2018, Nature Communications.

Pathophysiology and fate of hepatocytes in a mouse model of mitochondrial hepatopathies

A. Regev, J. Oca-Cossio, C. Moraes, 2007, Gut.

Mitochondrial gene editing

C. Moraes, S. Bacman, Jose Domingo Barrera-Paez, 2023, Nature Reviews Methods Primers.

Mitochondrial disorders caused by mutations in respiratory chain assembly factors.

V. Fellman, C. Moraes, F. Diaz, 2011, Seminars in fetal & neonatal medicine.

Overexpression of PGC‐1α in aging muscle enhances a subset of young‐like molecular patterns

C. Moraes, S. Garcia, F. Diaz, 2018, Aging cell.

Mitochondrial DNA deletion in a girl with manifestations of Kearns-Sayre and Lowe syndromes: an example of phenotypic mimicry?

S. Dimauro, E. Schon, M. Zeviani, 1991, American journal of medical genetics.

Extended polyglutamine repeats trigger a feedback loop involving the mitochondrial complex III, the proteasome and huntingtin aggregates.

H. Fukui, C. Moraes, 2007, Human molecular genetics.

Transcription and translation of deleted mitochondrial genomes in Kearns-Sayre syndrome: implications for pathogenesis.

S. Dimauro, E. Schon, R. Rizzuto, 1990, American journal of human genetics.

Distribution of wild-type and common deletion forms of mtDNA in normal and respiration-deficient muscle fibers from patients with mitochondrial myopathy.

S. Dimauro, E. Schon, C. Moraes, 1994, Human molecular genetics.

L-Arginine Reduces Nitro-Oxidative Stress in Cultured Cells with Mitochondrial Deficiency

C. Moraes, E. Higa, M. Mouro, 2021, Nutrients.

Treatment with ROS detoxifying gold quantum clusters alleviates the functional decline in a mouse model of Friedreich ataxia

G. Merlo, C. Riganti, C. Villa, 2021, Science Translational Medicine.

Cytochrome c Oxidase Is Required for the Assembly/Stability of Respiratory Complex I in Mouse Fibroblasts

H. Fukui, C. Moraes, S. Garcia, 2006, Molecular and Cellular Biology.

mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcription.

A. Torraco, C. Moraes, C. Luca, 2009, Cell metabolism.

Replication-competent human mitochondrial DNA lacking the heavy-strand promoter region

S. Dimauro, E. Schon, C. Moraes, 1991, Molecular and cellular biology.

Heterologous mitochondrial DNA recombination in human cells.

William M. Mauck, C. Moraes, G. Lenaz, 2004, Human molecular genetics.

Atypical clinical presentations associated with the MELAS mutation at position 3243 of human mitochondrial DNA

S. Dimauro, E. Schon, G. Silvestri, 1993, Neuromuscular Disorders.

Mouse models of Parkinson's disease associated with mitochondrial dysfunction

C. Moraes, Alicia M. Pickrell, M. Pinto, 2013, Molecular and Cellular Neuroscience.

Ablation of Cytochrome c in Adult Forebrain Neurons Impairs Oxidative Phosphorylation Without Detectable Apoptosis

C. Moraes, F. Diaz, U. Vempati, 2018, Molecular Neurobiology.

Phosphorylation of Cytochrome c Threonine 28 Regulates Electron Transport Chain Activity in Kidney

B. Edwards, T. Stemmler, T. Sanderson, 2016, The Journal of Biological Chemistry.

An out‐of‐frame cytochrome b gene deletion from a patient with parkinsonism is associated with impaired complex III assembly and an increase in free radical production

C. Moraes, F. Diaz, I. D. de Coo, 2000, Annals of neurology.

Elimination of Mutant Mitochondrial DNA in Mitochondrial Myopathies Using Gene-Editing Enzymes

C. Moraes, S. Bacman, 2019, Muscle Gene Therapy.

Sustained AMPK activation improves muscle function in a mitochondrial myopathy mouse model by promoting muscle fiber regeneration.

C. Moraes, S. Garcia, F. Diaz, 2016, Human molecular genetics.

Biallelic loss-of-function variations in PRDX3 cause cerebellar ataxia.

D. Timmann, S. Züchner, A. Durr, 2021, Brain : a journal of neurology.

Regional susceptibilities to mitochondrial dysfunctions in the CNS

C. Moraes, Alicia M. Pickrell, M. Pinto, 2012, Biological chemistry.

mitoTev‐TALE: a monomeric DNA editing enzyme to reduce mutant mitochondrial DNA levels

C. Moraes, S. Williams, S. Bacman, 2018, EMBO molecular medicine.

MitoTALEN: A General Approach to Reduce Mutant mtDNA Loads and Restore Oxidative Phosphorylation Function in Mitochondrial Diseases.

Marni J. Falk, M. Falk, D. Chan, 2015, Molecular therapy : the journal of the American Society of Gene Therapy.

Duplication and triplication with staggered breakpoints in human mitochondrial DNA.

C. Moraes, C. Tengan, 1998, Biochimica et biophysica acta.

Mitochondrial DNA Double-Strand Breaks in Oligodendrocytes Cause Demyelination, Axonal Injury, and CNS Inflammation

Kenji F. Tanaka, M. Taherian, C. Moraes, 2017, The Journal of Neuroscience.

Manipulating mitochondrial DNA heteroplasmy by a mitochondrially targeted restriction endonuclease.

S. Srivastava, C. Moraes, 2001, Human molecular genetics.

Suppression of a mitochondrial tRNA gene mutation phenotype associated with changes in the nuclear background.

C. Moraes, L. Morrison, H. Hao, 1999, Human molecular genetics.

Striatal Dysfunctions Associated with Mitochondrial DNA Damage in Dopaminergic Neurons in a Mouse Model of Parkinson's Disease

C. Moraes, Alicia M. Pickrell, Aline Hida, 2011, The Journal of Neuroscience.

Defects in the biosynthesis of mitochondrial heme c and heme a in yeast and mammals.

C. Moraes, A. Barrientos, F. Diaz, 2004, Biochimica et biophysica acta.

Mitochondrial DNA defects in Brazilian patients with chronic progressive external ophthalmoplegia

C. Moraes, A. Gabbai, C. Tengan, 1997, Journal of the Neurological Sciences.

Phenotype–genotype correlations in skeletal muscle of patients with mtDNA deletions

S. Dimauro, E. Schon, E. Ricci, 1995, Muscle & nerve. Supplement.

Superoxide released into the mitochondrial matrix.

D. Meany, E. Arriaga, C. Moraes, 2006, Free radical biology & medicine.

New Morphological Approaches to the Study of Mitochondrial Encephalomyopathies

V. Petruzzella, C. Moraes, E. Bonilla, 1992, Brain pathology.

Functional and Molecular Mitochondrial Abnormalities Associated with a C T Transition at Position 3256 of the Human Mitochondrial Genome

C. Moraes, H. Hao, 1996, The Journal of Biological Chemistry.

MitoTALENs for mtDNA editing

C. Moraes, S. Bacman, 2020 .

Up-regulation of D3 dopamine receptor mRNA in the nucleus accumbens of human cocaine fatalities.

D. Mash, D. Segal, C. Moraes, 1997, Brain research. Molecular brain research.

Correction: Somatic mtDNA Mutation Spectra in the Aging Human Putamen

D. Mash, S. Züchner, C. Moraes, 2013, PLoS Genetics.

High levels of mitochondrial DNA with an unstable 260-bp duplication in a patient with a mitochondrial myopathy

S. Dimauro, E. Schon, S. Servidei, 1995, Neurology.

Mitochondrial targeted meganuclease as a platform to eliminate mutant mtDNA in vivo

C. Moraes, D. Jantz, Jeff Smith, 2021, Nature Communications.

Disorders Associated with Depletion of Mitochondrial DNA

S. Dimauro, P. Tonali, E. Ricci, 1992, Brain pathology.

Rho(0) tumor cells: a model for studying whether mitochondria are targets for rhodamine 123, doxorubicin, and other drugs.

N. Savaraj, T. Lampidis, C. Moraes, 2000, Biochemical pharmacology.

Nitric Oxide Synthesis Is Increased in Cybrid Cells with m.3243A>G Mutation

C. Moraes, C. Tengan, B. H. Kiyomoto, 2012, International journal of molecular sciences.

Erratum: Increased muscle pgc-1αexpression protects from sarcopenia and metabolic disease during aging (Proceedings of the National Academy of Sciences of the United States of America (2009) 106 (20405-20410))

B. Spiegelman, C. Moraes, T. Wenz, 2014 .

Systematic association studies of mitochondrial DNA variations in schizophrenia: focus on the ND5 gene.

S. Manuck, M. Talkowski, R. Ferrell, 2008, Schizophrenia bulletin.

Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency

M. Baumgartner, D. di Bernardo, H. Debaix, 2020, Nature Communications.

Fatal infantile liver failure associated with mitochondrial DNA depletion.

E. Bertini, E. Ricci, S. Servidei, 1992, The Journal of pediatrics.

The use of mitochondria-targeted endonucleases to manipulate mtDNA.

C. Moraes, S. Williams, S. Bacman, 2014, Methods in enzymology.

Mitochondrial dysfunction characterized in human induced pluripotent stem cell disease models in MELAS syndrome: A brief summary.

C. Moraes, Kumarie Latchman, Mario Saporta, 2023, Mitochondrion.

Detection and analysis of mitochondrial DNA deletions by whole genome PCR.

C. Moraes, C. Tengan, 1996, Biochemical and molecular medicine.

Deletions of mitochondrial DNA in Kearns‐Sayre syndrome

S. Dimauro, E. Schon, M. Zeviani, 1988, Neurology.

Dopamine transporter mRNA in autopsy studies of chronic cocaine users.

D. Mash, D. Segal, C. Moraes, 1999, Brain research. Molecular brain research.

Glycolytic oligodendrocytes maintain myelin and long-term axonal integrity

J. Frahm, K. Nave, W. Möbius, 2012, Nature.

The Qo site of the mitochondrial complex III is required for the transduction of hypoxic signaling via reactive oxygen species production

J. Eisenbart, N. Chandel, C. Moraes, 2007, The Journal of cell biology.