Sambri, Irene
Sambri, Irene
A highly secreted sulphamidase engineered to cross the blood-brain barrier corrects brain lesions of mice with mucopolysaccharidoses type IIIA.
2013-01-01 Sorrentino, Nc; D'Orsi, L; Sambri, I; Nusco, E; Monaco, C; Spampanato, C; Polishchuk, E; Saccone, P; De Leonibus, E; Ballabio, A; Fraldi, A.
A Zebrafish Model to study RRAGD variants associated Cardiomyopathy.
2024-01-01 Sambri, I; Trepiccione, F.
Current methods to analyse lysosome morphology, positioning, motility and function
2022-01-01 Barral, Duarte C; Staiano, Leopoldo; Almeida, Cláudia Guimas; Cutler, Dan F; Eden, Emily R; Futter, Clare E; Galione, Antony; Marques, André R A; Medina, Diego Luis; Napolitano, Gennaro; Settembre, Carmine; Vieira, Otília V; Aerts, Johannes M F G; Atakpa-Adaji, Peace; Bruno, Gemma; Capuozzo, Antonella; De Leonibus, Elvira; Di Malta, Chiara; Escrevente, Cristina; Esposito, Alessandra; Grumati, Paolo; Hall, Michael J; Teodoro, Rita O; Lopes, Susana S; Luzio, J Paul; Monfregola, Jlenia; Montefusco, Sandro; Platt, Frances M; Polishuck, Roman; De Risi, Maria; Sambri, Irene; Soldati, Chiara; Seabra, Miguel C
Current methods to analyse lysosome morphology, positioning, motility and function
2022-01-01 Barral, Duarte C; Staiano, Leopoldo; Almeida, Cláudia Guimas; Cutler, Dan F; Eden, Emily R; Futter, Clare E; Galione, Antony; Marques, André R A; Medina, Diego Luis; Napolitano, Gennaro; Settembre, Carmine; Vieira, Otília V; Aerts, Johannes M F G; Atakpa-Adaji, Peace; Bruno, Gemma; Capuozzo, Antonella; De Leonibus, Elvira; Di Malta, Chiara; Escrevente, Cristina; Esposito, Alessandra; Grumati, Paolo; Hall, Michael J; Teodoro, Rita O; Lopes, Susana S; Luzio, J Paul; Monfregola, Jlenia; Montefusco, Sandro; Platt, Frances M; Polishuck, Roman; De Risi, Maria; Sambri, Irene; Soldati, Chiara; Seabra, Miguel C
Impaired flickering of the permeability transition pore causes SPG7 spastic paraplegia.
2020-01-01 Sambri, I; Massa, F; Gullo, F; Meneghini, S; Cassina, L; Carraro, M; Dina, G; Quattrini, A; Patanella, L; Carissimo, A; Iuliano, A; Santorelli, F; Codazzi, F; Grohovaz, F; Bernardi, P; Becchetti, A; Casari, G.
Lysosomal dysfunction disrupts presynaptic maintenance and restoration of presynaptic function prevents neurodegeneration in lysosomal storage diseases.
2017-01-01 Sambri, I; D'Alessio, R; Ezhova, Y; Giuliano, T; Sorrentino, Nc; Cacace, V; De Risi, M; Cataldi, M; Annunziato, L; De Leonibus, E; Fraldi, A.
Lysosomal dysfunction in neurodegenerative diseases
2015-01-01 Sambri, I; Fraldi, A
m-AAA and i-AAA complexes coordinate to regulate OMA1, the stress-activated supervisor of mitochondrial dynamics.
2018-01-01 Consolato, F; Maltecca, F; Tulli, S; Sambri, I; Casari, G.
miR-17 and -20a Target the Neuron-Derived Orphan Receptor-1 (NOR-1) in Vascular Endothelial Cells.
2015-01-01 Sambri, I; Crespo, J; Aguiló, S; Ingrosso, D; Rodríguez, C; Martínez González, J.
RagD auto-activating mutations impair MiT/TFE activity in kidney tubulopathy and cardiomyopathy syndrome
2023-01-01 Sambri, Irene; Ferniani, Marco; Campostrini, Giulia; Testa, Marialuisa; Meraviglia, Viviana; de Araujo, Mariana E G; Dokládal, Ladislav; Vilardo, Claudia; Monfregola, Jlenia; Zampelli, Nicolina; Vecchio Blanco, Francesca Del; Torella, Annalaura; Ruosi, Carolina; Fecarotta, Simona; Parenti, Giancarlo; Staiano, Leopoldo; Bellin, Milena; Huber, Lukas A; De Virgilio, Claudio; Trepiccione, Francesco; Nigro, Vincenzo; Ballabio, Andrea
Ragopathies and the rising influence of RagGTPases on human diseases.
2024-01-01 Sambri, I; Ferniani, M; Ballabio, A.
RRAGD-Associated Autosomal Dominant Kidney Hypomagnesemia with Cardiomyopathy: A Review on the Clinical Manifestations and Therapeutic Options.
2024-01-01 Trepiccione, F; Sambri, I; Ruggiero, B; Emma, F; Ballabio, A; Florio, G; Vanderheyden, I; Iervolino, A; Jouret, F
The Amyloid Inhibitor CLR01 Relieves Autophagy and Ameliorates Neuropathology in a Severe Lysosomal Storage Disease.
2020-01-01 Monaco, A; Maffia, V; Sorrentino, Nc; Sambri, I; Ezhova, Y; Giuliano, T; Cacace, V; Nusco, E; De Risi, M; De Leonibus, E; Schrader, T; Klärner, Fg; Bitan, G; Fraldi, A.
The molecular Tweezer CLR01 inhibits amyloid aggregation relieving lysosomal pathology and protecting against neurodegeneration in Lysosomal Storage Diseases
2019-01-01 Monaco, A; Maffia, V; Sorrentino, C; Ezhova, Y; Sambri, I; Cacace, V; Nusco, E; De Leonibus, E; Fraldi, A
The SGLT2 inhibitor dapagliflozin improves kidney function in glycogen storage disease XI.
2023-01-01 Trepiccione, F; Iervolino, A; D'Acierno, M; Siccardi, S; Costanzo, V; Sardella, D; De La Motte, Lr; D'Apolito, L; Miele, A; Perna, Af; Capolongo, G; Zacchia, M; Frische, S; Nielsen, R; Staiano, L; Sambri, I; De Cegli, R; Unwin, R; Eladari, D; Capasso, G.
Treatment of lysosomal storage disorders
2021-01-01 Bitan, G; Fraldi, A; Sambri, I; Monaco, A
| Titolo | Data di pubblicazione | Autore(i) | File |
|---|---|---|---|
| A highly secreted sulphamidase engineered to cross the blood-brain barrier corrects brain lesions of mice with mucopolysaccharidoses type IIIA. | 1-gen-2013 | Sorrentino, Nc; D'Orsi, L; Sambri, I; Nusco, E; Monaco, C; Spampanato, C; Polishchuk, E; Saccone, P; De Leonibus, E; Ballabio, A; Fraldi, A. | |
| A Zebrafish Model to study RRAGD variants associated Cardiomyopathy. | 1-gen-2024 | Sambri, I; Trepiccione, F. | |
| Current methods to analyse lysosome morphology, positioning, motility and function | 1-gen-2022 | Barral, Duarte C; Staiano, Leopoldo; Almeida, Cláudia Guimas; Cutler, Dan F; Eden, Emily R; Futter, Clare E; Galione, Antony; Marques, André R A; Medina, Diego Luis; Napolitano, Gennaro; Settembre, Carmine; Vieira, Otília V; Aerts, Johannes M F G; Atakpa-Adaji, Peace; Bruno, Gemma; Capuozzo, Antonella; De Leonibus, Elvira; Di Malta, Chiara; Escrevente, Cristina; Esposito, Alessandra; Grumati, Paolo; Hall, Michael J; Teodoro, Rita O; Lopes, Susana S; Luzio, J Paul; Monfregola, Jlenia; Montefusco, Sandro; Platt, Frances M; Polishuck, Roman; De Risi, Maria; Sambri, Irene; Soldati, Chiara; Seabra, Miguel C | |
| Current methods to analyse lysosome morphology, positioning, motility and function | 1-gen-2022 | Barral, Duarte C; Staiano, Leopoldo; Almeida, Cláudia Guimas; Cutler, Dan F; Eden, Emily R; Futter, Clare E; Galione, Antony; Marques, André R A; Medina, Diego Luis; Napolitano, Gennaro; Settembre, Carmine; Vieira, Otília V; Aerts, Johannes M F G; Atakpa-Adaji, Peace; Bruno, Gemma; Capuozzo, Antonella; De Leonibus, Elvira; Di Malta, Chiara; Escrevente, Cristina; Esposito, Alessandra; Grumati, Paolo; Hall, Michael J; Teodoro, Rita O; Lopes, Susana S; Luzio, J Paul; Monfregola, Jlenia; Montefusco, Sandro; Platt, Frances M; Polishuck, Roman; De Risi, Maria; Sambri, Irene; Soldati, Chiara; Seabra, Miguel C | |
| Impaired flickering of the permeability transition pore causes SPG7 spastic paraplegia. | 1-gen-2020 | Sambri, I; Massa, F; Gullo, F; Meneghini, S; Cassina, L; Carraro, M; Dina, G; Quattrini, A; Patanella, L; Carissimo, A; Iuliano, A; Santorelli, F; Codazzi, F; Grohovaz, F; Bernardi, P; Becchetti, A; Casari, G. | |
| Lysosomal dysfunction disrupts presynaptic maintenance and restoration of presynaptic function prevents neurodegeneration in lysosomal storage diseases. | 1-gen-2017 | Sambri, I; D'Alessio, R; Ezhova, Y; Giuliano, T; Sorrentino, Nc; Cacace, V; De Risi, M; Cataldi, M; Annunziato, L; De Leonibus, E; Fraldi, A. | |
| Lysosomal dysfunction in neurodegenerative diseases | 1-gen-2015 | Sambri, I; Fraldi, A | |
| m-AAA and i-AAA complexes coordinate to regulate OMA1, the stress-activated supervisor of mitochondrial dynamics. | 1-gen-2018 | Consolato, F; Maltecca, F; Tulli, S; Sambri, I; Casari, G. | |
| miR-17 and -20a Target the Neuron-Derived Orphan Receptor-1 (NOR-1) in Vascular Endothelial Cells. | 1-gen-2015 | Sambri, I; Crespo, J; Aguiló, S; Ingrosso, D; Rodríguez, C; Martínez González, J. | |
| RagD auto-activating mutations impair MiT/TFE activity in kidney tubulopathy and cardiomyopathy syndrome | 1-gen-2023 | Sambri, Irene; Ferniani, Marco; Campostrini, Giulia; Testa, Marialuisa; Meraviglia, Viviana; de Araujo, Mariana E G; Dokládal, Ladislav; Vilardo, Claudia; Monfregola, Jlenia; Zampelli, Nicolina; Vecchio Blanco, Francesca Del; Torella, Annalaura; Ruosi, Carolina; Fecarotta, Simona; Parenti, Giancarlo; Staiano, Leopoldo; Bellin, Milena; Huber, Lukas A; De Virgilio, Claudio; Trepiccione, Francesco; Nigro, Vincenzo; Ballabio, Andrea | |
| Ragopathies and the rising influence of RagGTPases on human diseases. | 1-gen-2024 | Sambri, I; Ferniani, M; Ballabio, A. | |
| RRAGD-Associated Autosomal Dominant Kidney Hypomagnesemia with Cardiomyopathy: A Review on the Clinical Manifestations and Therapeutic Options. | 1-gen-2024 | Trepiccione, F; Sambri, I; Ruggiero, B; Emma, F; Ballabio, A; Florio, G; Vanderheyden, I; Iervolino, A; Jouret, F | |
| The Amyloid Inhibitor CLR01 Relieves Autophagy and Ameliorates Neuropathology in a Severe Lysosomal Storage Disease. | 1-gen-2020 | Monaco, A; Maffia, V; Sorrentino, Nc; Sambri, I; Ezhova, Y; Giuliano, T; Cacace, V; Nusco, E; De Risi, M; De Leonibus, E; Schrader, T; Klärner, Fg; Bitan, G; Fraldi, A. | |
| The molecular Tweezer CLR01 inhibits amyloid aggregation relieving lysosomal pathology and protecting against neurodegeneration in Lysosomal Storage Diseases | 1-gen-2019 | Monaco, A; Maffia, V; Sorrentino, C; Ezhova, Y; Sambri, I; Cacace, V; Nusco, E; De Leonibus, E; Fraldi, A | |
| The SGLT2 inhibitor dapagliflozin improves kidney function in glycogen storage disease XI. | 1-gen-2023 | Trepiccione, F; Iervolino, A; D'Acierno, M; Siccardi, S; Costanzo, V; Sardella, D; De La Motte, Lr; D'Apolito, L; Miele, A; Perna, Af; Capolongo, G; Zacchia, M; Frische, S; Nielsen, R; Staiano, L; Sambri, I; De Cegli, R; Unwin, R; Eladari, D; Capasso, G. | |
| Treatment of lysosomal storage disorders | 1-gen-2021 | Bitan, G; Fraldi, A; Sambri, I; Monaco, A |