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E. Nusco · SciRah

Researcher profile · Semantic Scholar

E. Nusco

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E. Nusco — researcher profile on SciRah

h-index: 27
Works: 59
Citations: 2,390

Most-cited papers

A substrate-specific mTORC1 pathway underlies Birt-Hogg-Dubé syndrome
G. Napolitano, Chiara Di Malta, Alessandra Esposito, Mariana E. G. de Araujo +16 more
2020Nature292 citations Open access
Semantic Scholar
TRANSCRIPTIONAL ACTIVATION OF RAGD GTPASE CONTROLS mTORC1 AND PROMOTES CANCER GROWTH
Chiara Di Malta, Diletta Siciliano, Alessia Calcagni', J. Monfregola +17 more
2017Science217 citations Open access
Semantic Scholar
FGF signalling regulates bone growth through autophagy
Laura Cinque, A. Forrester, Rosa Bartolomeo, M. Svelto +9 more
2015Nature203 citations
Semantic Scholar
A highly secreted sulphamidase engineered to cross the blood-brain barrier corrects brain lesions of mice with mucopolysaccharidoses type IIIA
N. Sorrentino, Luca D'Orsi, Irene Sambri, E. Nusco +7 more
2013EMBO Molecular Medicine109 citations Open access
Semantic Scholar
Systemic inflammation and neurodegeneration in a mouse model of multiple sulfatase deficiency
C. Settembre, Ida Annunziata, C. Spampanato, D. Zarcone +6 more
2007Proceedings of the National Academy of Sciences of the United States of America103 citations Open access
Semantic Scholar
Pyruvate dehydrogenase complex and lactate dehydrogenase are targets for therapy of acute liver failure
R. Ferriero, E. Nusco, R. De Cegli, A. Carissimo +2 more
2018Journal of Hepatology96 citations Open access
Semantic Scholar
Modelling TFE renal cell carcinoma in mice reveals a critical role of WNT signaling
Alessia Calcagni', L. Kors, Eric H. J. Verschuren, R. De Cegli +13 more
2016eLife91 citations Open access
Semantic Scholar
mTORC1 hyperactivation arrests bone growth in lysosomal storage disorders by suppressing autophagy.
Rosa Bartolomeo, Laura Cinque, C. De Leonibus, A. Forrester +10 more
2017Journal of Clinical Investigation90 citations Open access
Semantic Scholar
A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.
G. Parenti, Simona Fecarotta, G. la Marca, Barbara Rossi +18 more
2014Molecular Therapy86 citations Open access
Semantic Scholar
SUMF1 enhances sulfatase activities in vivo in five sulfatase deficiencies.
A. Fraldi, A. Biffi, A. Lombardi, I. Visigalli +7 more
2007Biochemical Journal78 citations Open access
Semantic Scholar
Phenylbutyrate Therapy for Pyruvate Dehydrogenase Complex Deficiency and Lactic Acidosis
R. Ferriero, Giuseppe Manco, E. Lamantea, E. Nusco +6 more
2013Science Translational Medicine70 citations Open access
Semantic Scholar
Enhancement of hepatic autophagy increases ureagenesis and protects against hyperammonemia
Leandro R. Soria, Gabriella Allegri, D. Melck, N. Pastore +9 more
2017Proceedings of the National Academy of Sciences of the United States of America59 citations Open access
Semantic Scholar
TFEB and TFE3 drive kidney cystogenesis and tumorigenesis
Chiara Di Malta, Angela Zampelli, L. Granieri, C. Vilardo +19 more
2023EMBO Molecular Medicine54 citations Open access
Semantic Scholar
Impaired parkin-mediated mitochondrial targeting to autophagosomes differentially contributes to tissue pathology in lysosomal storage diseases
Raquel de Pablo-Latorre, Assunta Saide, Elena V. Polishhuck, E. Nusco +2 more
2012Human Molecular Genetics52 citations Open access
Semantic Scholar
Tbx1 regulates brain vascularization.
S. Cioffi, S. Martucciello, F. G. Fulcoli, M. Bilio +3 more
2014Human Molecular Genetics49 citations Open access
Semantic Scholar
Low incidence of hepatocellular carcinoma in mice and cats treated with systemic adeno-associated viral vectors
R. Ferla, M. Alliegro, Margherita Dell’Anno, E. Nusco +13 more
2020Molecular Therapy: Methods & Clinical Development47 citations Open access
Semantic Scholar
Non-clinical Safety and Efficacy of an AAV2/8 Vector Administered Intravenously for Treatment of Mucopolysaccharidosis Type VI
R. Ferla, M. Alliegro, J. Marteau, Margherita Dell’Anno +6 more
2017Molecular Therapy: Methods & Clinical Development45 citations Open access
Semantic Scholar
Correction of CNS defects in the MPSII mouse model via systemic enzyme replacement therapy
V. A. Polito, Serena Abbondante, R. Polishchuk, E. Nusco +2 more
2010Human Molecular Genetics44 citations Open access
Semantic Scholar
AAV-mediated transcription factor EB (TFEB) gene delivery ameliorates muscle pathology and function in the murine model of Pompe Disease
F. Gatto, Barbara Rossi, Antonietta Tarallo, Elena Polishchuk +10 more
2017Scientific Reports42 citations Open access
Semantic Scholar
Efficacy of a combined intracerebral and systemic gene delivery approach for the treatment of a severe lysosomal storage disorder.
C. Spampanato, E. De Leonibus, Paola Dama, A. Gargiulo +7 more
2011Molecular Therapy42 citations Open access
Semantic Scholar
Intracranial gene delivery of LV‐NAGLU vector corrects neuropathology in murine MPS IIIB
C. di Domenico, G. Villani, D. Di Napoli, E. Nusco +3 more
2009American Journal of Medical Genetics. Part A42 citations
Semantic Scholar
Therapeutic homology-independent targeted integration in retina and liver
P. Tornabene, R. Ferla, Manel Llado-Santaeularia, M. Centrulo +19 more
2022Nature Communications39 citations Open access
Semantic Scholar
The Amyloid Inhibitor CLR01 Relieves Autophagy and Ameliorates Neuropathology in a Severe Lysosomal Storage Disease
A. Monaco, Veronica Maffia, N. Sorrentino, Irene Sambri +10 more
2020Molecular Therapy36 citations Open access
Semantic Scholar
Light‐responsive microRNA miR‐211 targets Ezrin to modulate lysosomal biogenesis and retinal cell clearance
Federica Naso, Daniela Intartaglia, Danila Falanga, C. Soldati +16 more
2020EMBO Journal36 citations Open access
Semantic Scholar
microRNAs as biomarkers in Pompe disease
Antonietta Tarallo, A. Carissimo, F. Gatto, E. Nusco +21 more
2018Genetics in Medicine33 citations Open access
Semantic Scholar
Disease Rescue and Increased Lifespan in a Model of Cardiomyopathy and Muscular Dystrophy by Combined AAV Treatments
C. Vitiello, Stefania Faraso, N. Sorrentino, G. di Salvo +6 more
2009PLoS ONE28 citations Open access
Semantic Scholar
Full-length ATP7B reconstituted through protein trans-splicing corrects Wilson disease in mice
Agnese Padula, Raffaella Petruzzelli, Sasha A. Philbert, Stephanie J Church +11 more
2022Molecular Therapy: Methods & Clinical Development27 citations Open access
Semantic Scholar
Safe and effective liver-directed AAV-mediated homology-independent targeted integration in mouse models of inherited diseases
F. Esposito, Fabio Dell'aquila, M. Rhiel, Stefano Auricchio +19 more
2024Cell Reports Medicine26 citations Open access
Semantic Scholar
O-GlcNAcylation enhances CPS1 catalytic efficiency for ammonia and promotes ureagenesis
Leandro R. Soria, Georgios Makris, A. D'Alessio, Angela de Angelis +18 more
2022Nature Communications26 citations Open access
Semantic Scholar
Impact of liver fibrosis on AAV-mediated gene transfer to mouse hepatocytes
R. Ferriero, Gemma Bruno, Agnese Padula, Simone Pisano +20 more
2025Nature Communications23 citations Open access
Semantic Scholar
Correction of oxidative stress enhances enzyme replacement therapy in Pompe disease
Antonietta Tarallo, C. Damiano, Sandra Strollo, Nadia Minopoli +15 more
2021EMBO Molecular Medicine22 citations Open access
Semantic Scholar
CHOP and c-JUN up-regulate the mutant Z α1-antitrypsin, exacerbating its aggregation and liver proteotoxicity
S. Attanasio, R. Ferriero, Gwladys Gernoux, R. De Cegli +7 more
2020Journal of Biological Chemistry20 citations Open access
Semantic Scholar
Liver‐directed gene therapy for ornithine aminotransferase deficiency
I. Boffa, Elena Polishchuk, L. De Stefano, Fabio Dell'aquila +11 more
2023EMBO Molecular Medicine19 citations Open access
Semantic Scholar
Retinal Degeneration in MPS-IIIA Mouse Model
Daniela Intartaglia, Giuliana Giamundo, E. Marrocco, Veronica Maffia +5 more
2020Frontiers in Cell and Developmental Biology19 citations Open access
Semantic Scholar
TFEB controls syncytiotrophoblast formation and hormone production in placenta
M. Cesana, Gennaro Tufano, F. Panariello, Nicolina Zampelli +17 more
2024Cell Death and Differentiation18 citations Open access
Semantic Scholar
Genome editing without nucleases confers proliferative advantage to edited hepatocytes and corrects Wilson disease
Agnese Padula, M. Spinelli, E. Nusco, Xabier Bujanda Cundin +10 more
2023JCI Insight15 citations Open access
Semantic Scholar
Enhancing the Therapeutic Potential of Sulfamidase for the Treatment of Mucopolysaccharidosis IIIA
N. Sorrentino, Vincenzo Cacace, M. D. de Risi, Veronica Maffia +11 more
2019Molecular Therapy: Methods & Clinical Development14 citations Open access
Semantic Scholar
Low-dose Gene Therapy Reduces the Frequency of Enzyme Replacement Therapy in a Mouse Model of Lysosomal Storage Disease
M. Alliegro, R. Ferla, E. Nusco, C. De Leonibus +2 more
2016Molecular Therapy14 citations Open access
Semantic Scholar
Liver gene therapy with intein‐mediated F8 trans‐splicing corrects mouse haemophilia A
F. Esposito, Hristiana Lyubenova, P. Tornabene, Stefano Auricchio +9 more
2022EMBO Molecular Medicine12 citations Open access
Semantic Scholar
Induction of Autophagy Promotes Clearance of RHOP23H Aggregates and Protects From Retinal Degeneration
Daniela Intartaglia, Giuliana Giamundo, Federica Naso, E. Nusco +4 more
2022Frontiers in Aging Neuroscience12 citations Open access
Semantic Scholar
TFEB coordinates autophagosome biogenesis and ribophagy during starvation via SQSTM1
Maria Iavazzo, Laura Cinque, Sophie Levantovsky, Castrese Morrone +11 more
2026Science Advances11 citations Open access
Semantic Scholar
Improved Efficacy and Reduced Toxicity by Ultrasound-Guided Intrahepatic Injections of Helper-Dependent Adenoviral Vector in Gunn Rats
N. Pastore, E. Nusco, Pasquale Piccolo, S. Castaldo +6 more
2013Human Gene Therapy Methods10 citations
Semantic Scholar
Increased expression or activation of TRPML1 reduces hepatic storage of toxic Z alpha-1 antitrypsin.
N. Pastore, Francesco Annunziata, Rita Colonna, Veronica Maffia +10 more
2023Molecular Therapy9 citations Open access
Semantic Scholar
Sustained Reduction of Hyperbilirubinemia in Gunn Rats After Adeno-Associated Virus-Mediated Gene Transfer of Bilirubin UDP-Glucuronosyltransferase Isozyme 1A1 to Skeletal Muscle
N. Pastore, E. Nusco, Jana Vaníková, R. Sepe +5 more
2012Human Gene Therapy8 citations
Semantic Scholar
Hepatocyte delivery of miR-34b/c reduces hepatic stellate cell activation and improves liver fibrosis
Pasquale Piccolo, R. Ferriero, Claudia Perna, E. Nusco +9 more
2025Molecular Therapy: Nucleic Acids3 citations
Semantic Scholar
TRPML-1 Dysfunction and Renal Tubulopathy in Mucolipidosis Type IV
Giuseppina Grieco, S. Montefusco, E. Nusco, A. Capuozzo +16 more
2024Journal of the American Society of Nephrology3 citations Open access
Semantic Scholar
Combined intraocular and intravenous gene delivery for therapy of gyrate atrophy of the choroid and retina
Fabio Dell'aquila, Roberto Di Cunto, E. Marrocco, E. Del Prete +5 more
2024Molecular Therapy2 citations Open access
Semantic Scholar
CHOP-c-JUN complex plays a critical role in liver proteotoxicity induced by mutant Z alpha-1 antitrypsin
S. Attanasio, Gwladys Gernoux, R. Ferriero, R. De Cegli +7 more
2020bioRxiv2 citations Open access
Semantic Scholar
Hepatic autophagy potentiates ureagenesis and its enhancement protects against acute and chronic hyperammonemia
Leandro R. Soria, Gabriella Allegri, D. Melck, N. Pastore +9 more
20181 citations
Semantic Scholar
S-Nitrosylation of glycogen phosphorylase regulates nuclear glycogenolysis and is impaired in metabolic liver disorders
L. R. Soria, A. D'Alessio, Vincenzo Casertano, Elena Polishchuk +35 more
2026Science Advances0 citations Open access
Semantic Scholar

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