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C. Viscomi · SciRah

Researcher profile · Semantic Scholar

C. Viscomi

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C. Viscomi — researcher profile on SciRah

h-index: 47
Works: 141
Citations: 8,554

Most-cited papers

Niche stiffness underlies the aging of central nervous system progenitor cells
Michael Segel, Björn Neumann, M. Hill, Isabell P. Weber +12 more
2019Nature453 citations Open access
Semantic Scholar
The Opa1-Dependent Mitochondrial Cristae Remodeling Pathway Controls Atrophic, Apoptotic, and Ischemic Tissue Damage
T. Varanita, M. Soriano, V. Romanello, T. Zaglia +12 more
2015Cell Metabolism443 citations Open access
Semantic Scholar
NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease
R. Cerutti, E. Pirinen, C. Lamperti, S. Marchet +8 more
2014Cell Metabolism345 citations Open access
Semantic Scholar
Control of mitochondrial superoxide production by reverse electron transport at complex I
Ellen L. Robb, A. R. Hall, Tracy A. Prime, S. Eaton +4 more
2018Journal of Biological Chemistry299 citations Open access
Semantic Scholar
Transcription Factor EB Controls Metabolic Flexibility during Exercise
G. Mansueto, A. Armani, C. Viscomi, Luca D'Orsi +17 more
2017Cell Metabolism296 citations Open access
Semantic Scholar
Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo
P. Gammage, C. Viscomi, M. Simard, Ana S. H. Costa +12 more
2018Nature Medicine273 citations Open access
Semantic Scholar
Pharmacological Inhibition of Poly(ADP-Ribose) Polymerases Improves Fitness and Mitochondrial Function in Skeletal Muscle
E. Pirinen, C. Cantó, Y. Jo, L. Morató +18 more
2014Cell Metabolism254 citations Open access
Semantic Scholar
Neural stem cells traffic functional mitochondria via extracellular vesicles
L. Peruzzotti-Jametti, J. Bernstock, Cory M. Willis, Giulia Manferrari +26 more
2021PLoS Biology216 citations Open access
Semantic Scholar
Opa1 Overexpression Ameliorates the Phenotype of Two Mitochondrial Disease Mouse Models
G. Civiletto, T. Varanita, R. Cerutti, Tatiana Gorletta +6 more
2015Cell Metabolism215 citations Open access
Semantic Scholar
FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders
Jenni M. Lehtonen, S. Forsström, Emanuela Bottani, C. Viscomi +20 more
2016Neurology203 citations Open access
Semantic Scholar
CryoEM structures of complex I from mouse heart mitochondria in two biochemically-defined states
Ahmed-Noor A. Agip, J. Blaza, H. R. Bridges, C. Viscomi +3 more
2018Nature Structural & Molecular Biology186 citations Open access
Semantic Scholar
MtDNA-maintenance defects: syndromes and genes
C. Viscomi, M. Zeviani
2017Journal of Inherited Metabolic Disease165 citations Open access
Semantic Scholar
Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer’s disease-like pathology in human cerebral organoids
M. Pérez, D. Ivanyuk, Vasiliki Panagiotakopoulou, G. Di Napoli +9 more
2020bioRxiv134 citations Open access
Semantic Scholar
Structural basis for a complex I mutation that blocks pathological ROS production
Z. Yin, N. Burger, Duvaraka Kula-Alwar, D. Aksentijević +9 more
2021Nature Communications123 citations Open access
Semantic Scholar
Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis
G. Civiletto, S. Dogan, R. Cerutti, G. Fagiolari +5 more
2018EMBO Molecular Medicine112 citations Open access
Semantic Scholar
Emerging concepts in the therapy of mitochondrial disease.
C. Viscomi, Emanuela Bottani, M. Zeviani
2015Biochimica et Biophysica Acta111 citations Open access
Semantic Scholar
Complex IV Deficient Surf1−/− Mice Initiate Mitochondrial Stress Responses
Daniel A. Pulliam, S. S. Deepa, Yu-Hong Liu, Shauna A. Hill +7 more
2014Biochemical Journal105 citations Open access
Semantic Scholar
Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy
S. Dogan, R. Cerutti, Cristiane Benincá, Gloria Brea-Calvo +4 more
2018Cell Metabolism91 citations Open access
Semantic Scholar
Metabolic effects of bezafibrate in mitochondrial disease
H. Steele, A. Gomez-Duran, A. Pyle, Sila Hopton +13 more
2020EMBO Molecular Medicine79 citations Open access
Semantic Scholar
TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.
Emanuela Bottani, R. Cerutti, M. Harbour, S. Ravaglia +7 more
2017Molecules and Cells76 citations Open access
Semantic Scholar
Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegeneration
D. Brunetti, Janniche Torsvik, C. Dallabona, P. Teixeira +17 more
2015EMBO Molecular Medicine69 citations Open access
Semantic Scholar
Gene therapy using a liver-targeted AAV vector restores nucleoside and nucleotide homeostasis in a murine model of MNGIE.
Javier Torres-Torronteras, C. Viscomi, R. Cabrera-Pérez, Yolanda Cámara +7 more
2014Molecular Therapy69 citations Open access
Semantic Scholar
AAV9-based gene therapy partially ameliorates the clinical phenotype of a mouse model of Leigh syndrome
I. Di Meo, S. Marchet, C. Lamperti, M. Zeviani +1 more
2017Gene Therapy60 citations Open access
Semantic Scholar
Defective endoplasmic reticulum-mitochondria contacts and bioenergetics in SEPN1-related myopathy
A. Filipe, A. Chernorudskiy, S. Arbogast, E. Varone +21 more
2020Cell Death and Differentiation57 citations Open access
Semantic Scholar
Bioenergetic consequences from xenotopic expression of a tunicate AOX in mouse mitochondria: Switch from RET and ROS to FET.
M. Szibor, T. Gainutdinov, E. Fernández-Vizarra, E. Dufour +7 more
2019Biochimica et Biophysica Acta - Bioenergetics54 citations Open access
Semantic Scholar
Strategies for fighting mitochondrial diseases
C. Viscomi, M. Zeviani
2020Journal of Internal Medicine53 citations Open access
Semantic Scholar
Towards a therapy for mitochondrial disease: an update
C. Garone, C. Viscomi
2018Biochemical Society Transactions53 citations Open access
Semantic Scholar
Long-Term Sustained Effect of Liver-Targeted Adeno-Associated Virus Gene Therapy for Mitochondrial Neurogastrointestinal Encephalomyopathy
Javier Torres-Torronteras, R. Cabrera-Pérez, Ferran Vila-Julià, C. Viscomi +4 more
2017Human Gene Therapy48 citations Open access
Semantic Scholar
Mitochondrial Neurodegeneration
M. Zeviani, C. Viscomi
2022Cells47 citations Open access
Semantic Scholar
A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4−/− Mice
Pedro Silva-Pinheiro, R. Cerutti, Marta Luna-Sánchez, M. Zeviani +1 more
2020Molecular Therapy: Methods & Clinical Development44 citations Open access
Semantic Scholar
Mitochondrial DNA heteroplasmy is modulated during oocyte development propagating mutation transmission
Hai-Xin Zhang, Marco Esposito, M. Pezet, J. Aryaman +11 more
2021Science Advances38 citations Open access
Semantic Scholar
Mouse mitochondrial complex I in the deactive state
Ahmed-Noor A. Agip, J. N. Blaza, H. R. Bridges, C. Viscomi +3 more
201838 citations
Semantic Scholar
Down-regulation of the mitochondrial aspartate-glutamate carrier isoform 1 AGC1 inhibits proliferation and N-acetylaspartate synthesis in Neuro2A cells.
E. Profilo, Luis Emiliano Peña-Altamira, M. Corricelli, Alessandra Castegna +18 more
2017Biochimica et Biophysica Acta - Molecular Basis of Disease35 citations Open access
Semantic Scholar
Gene therapy for primary mitochondrial diseases: experimental advances and clinical challenges
M. Falabella, M. Minczuk, M. Hanna, C. Viscomi +1 more
2022Nature Reviews Neurology34 citations
Semantic Scholar
Physical and Functional Cross Talk Between Endo-Sarcoplasmic Reticulum and Mitochondria in Skeletal Muscle
S. Boncompagni, D. Pozzer, C. Viscomi, A. Ferreiro +1 more
2019Antioxidants and Redox Signaling34 citations
Semantic Scholar
Mitochondrial respiratory states and rates
E. Gnaiger, E. A. Frostner, N. A. Karim, N. Abumrad +496 more
201933 citations Open access
Semantic Scholar
SURF1 knockout cloned pigs: Early onset of a severe lethal phenotype
C. Quadalti, D. Brunetti, I. Lagutina, R. Duchi +16 more
2018Biochimica et Biophysica Acta - Molecular Basis of Disease33 citations Open access
Semantic Scholar
Toward a therapy for mitochondrial disease
C. Viscomi
2016Biochemical Society Transactions33 citations Open access
Semantic Scholar
Role of PITRM1 in Mitochondrial Dysfunction and Neurodegeneration
D. Brunetti, Alessia Catania, C. Viscomi, M. Deleidi +3 more
2021Biomedicines32 citations Open access
Semantic Scholar
Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects
N. Kovářová, P. Pecina, H. Nůsková, M. Vrbacký +4 more
2016Biochimica et Biophysica Acta31 citations Open access
Semantic Scholar
Gene therapy for mitochondrial disorders
Nandaki Keshavan, M. Minczuk, C. Viscomi, Shamima Rahman
2024Journal of Inherited Metabolic Disease30 citations Open access
Semantic Scholar
DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit depletion
Pedro Silva-Pinheiro, Carlos Pardo-Hernández, A. Reyes, Lisa Tilokani +16 more
2021Nucleic Acids Research30 citations Open access
Semantic Scholar
Exploiting pyocyanin to treat mitochondrial disease due to respiratory complex III dysfunction
R. Peruzzo, Samantha Corrà, Roberto Costa, M. Brischigliaro +11 more
2021Nature Communications27 citations Open access
Semantic Scholar
APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS
Alba Signes, R. Cerutti, Anna S. Dickson, Cristiane Benincá +9 more
2018EMBO Molecular Medicine24 citations Open access
Semantic Scholar
Structural rather than catalytic role for mitochondrial respiratory chain supercomplexes
M. Brischigliaro, Alfredo Cabrera-Orefice, S. Arnold, C. Viscomi +2 more
2023bioRxiv23 citations Open access
Semantic Scholar
Measurement of mitochondrial respiratory chain enzymatic activities in Drosophila melanogaster samples
M. Brischigliaro, Elena Frigo, E. Fernández-Vizarra, P. Bernardi +1 more
2022STAR Protocols23 citations Open access
Semantic Scholar
Respiratory chain signalling is essential for adaptive remodelling following cardiac ischaemia
M. Szibor, R. Schreckenberg, Z. Gizatullina, E. Dufour +16 more
2020Journal of Cellular and Molecular Medicine21 citations Open access
Semantic Scholar
Mitochondrial Neurodegeneration: Lessons from Drosophila melanogaster Models
M. Brischigliaro, E. Fernández-Vizarra, C. Viscomi
2023Biomolecules18 citations Open access
Semantic Scholar
Xenotopic expression of alternative oxidase (AOX) to study mechanisms of mitochondrial disease.
C. Viscomi, A. Moore, M. Zeviani, M. Szibor
2022Biochimica et Biophysica Acta - Bioenergetics17 citations Open access
Semantic Scholar
Lifelong reduction in complex IV induces tissue‐specific metabolic effects but does not reduce lifespan or healthspan in mice
S. S. Deepa, Gavin A. Pharaoh, M. Kinter, Vivian Diaz +11 more
2018Aging Cell17 citations Open access
Semantic Scholar

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