Zobrazeno 1 - 10
of 74
pro vyhledávání: '"Diego L. Medina"'
Autor:
Irene Lopez-Fabuel, Marina Garcia-Macia, Costantina Buondelmonte, Olga Burmistrova, Nicolo Bonora, Paula Alonso-Batan, Brenda Morant-Ferrando, Carlos Vicente-Gutierrez, Daniel Jimenez-Blasco, Ruben Quintana-Cabrera, Emilio Fernandez, Jordi Llop, Pedro Ramos-Cabrer, Aseel Sharaireh, Marta Guevara-Ferrer, Lorna Fitzpatrick, Christopher D. Thompton, Tristan R. McKay, Stephan Storch, Diego L. Medina, Sara E. Mole, Peter O. Fedichev, Angeles Almeida, Juan P. Bolaños
Publikováno v:
Nature Communications, Vol 13, Iss 1, Pp 1-14 (2022)
CLN7 neuronal ceroid lipofuscinosis is an inherited lysosomal storage disease typically with childhood onset of neurodegenerative symptoms. Here the authors report that in a mouse model of CLN7 disease neuronal reactive oxygen species and the activit
Externí odkaz:
https://doaj.org/article/3a651b3190b84e729467d309cc661713
Autor:
Pablo S. Contreras, Pablo J. Tapia, Lila González-Hódar, Ivana Peluso, Chiara Soldati, Gennaro Napolitano, Maria Matarese, Macarena Las Heras, Cristian Valls, Alexis Martinez, Elisa Balboa, Juan Castro, Nancy Leal, Frances M. Platt, Andrzej Sobota, Dominic Winter, Andrés D. Klein, Diego L. Medina, Andrea Ballabio, Alejandra R. Alvarez, Silvana Zanlungo
Publikováno v:
iScience, Vol 23, Iss 11, Pp 101691- (2020)
Summary: The transcription factor EB (TFEB) has emerged as a master regulator of lysosomal biogenesis, exocytosis, and autophagy, promoting the clearance of substrates stored in cells. c-Abl is a tyrosine kinase that participates in cellular signalin
Externí odkaz:
https://doaj.org/article/c6a46857605147c4a1da866ca411c8d6
Autor:
Magdalena Węsierska, Anna Kloska, Diego L. Medina, Joanna Jakóbkiewicz-Banecka, Magdalena Gabig-Cimińska, Marta Radzińska, Marta Moskot, Marcelina Malinowska
Publikováno v:
International Journal of Molecular Sciences, Vol 23, Iss 3, p 1058 (2022)
Flavonoids are investigated as therapeutics for mucopolysaccharidosis, a metabolic disorder with impaired glycosaminoglycan degradation. Here we determined the effects of genistein and kaempferol, used alone or in combination, on cellular response an
Externí odkaz:
https://doaj.org/article/ae1ce8ecbeef425eaddc22216d165fbd
Autor:
Anna Scotto Rosato, Einar K Krogsaeter, Dawid Jaślan, Carla Abrahamian, Sandro Montefusco, Chiara Soldati, Barbara Spix, Maria Teresa Pizzo, Giuseppina Grieco, Julia Böck, Amanda Wyatt, Daniela Wünkhaus, Marcel Passon, Marc Stieglitz, Marco Keller, Guido Hermey, Sandra Markmann, Doris Gruber‐Schoffnegger, Susan Cotman, Ludger Johannes, Dennis Crusius, Ulrich Boehm, Christian Wahl‐Schott, Martin Biel, Franz Bracher, Elvira De Leonibus, Elena Polishchuk, Diego L Medina, Dominik Paquet, Christian Grimm
Publikováno v:
EMBO Molecular Medicine, Vol 14, Iss 9, Pp 1-17 (2022)
Abstract Lysosomes are cell organelles that degrade macromolecules to recycle their components. If lysosomal degradative function is impaired, e.g., due to mutations in lysosomal enzymes or membrane proteins, lysosomal storage diseases (LSDs) can dev
Externí odkaz:
https://doaj.org/article/81fb1739060044f693962704d333fab5
EGR1 drives cell proliferation by directly stimulating TFEB transcription in response to starvation.
Autor:
Marcella Cesana, Gennaro Tufano, Francesco Panariello, Nicolina Zampelli, Susanna Ambrosio, Rossella De Cegli, Margherita Mutarelli, Lorenzo Vaccaro, Micheal J Ziller, Davide Cacchiarelli, Diego L Medina, Andrea Ballabio
Publikováno v:
PLoS Biology, Vol 21, Iss 3, p e3002034 (2023)
The stress-responsive transcription factor EB (TFEB) is a master controller of lysosomal biogenesis and autophagy and plays a major role in several cancer-associated diseases. TFEB is regulated at the posttranslational level by the nutrient-sensitive
Externí odkaz:
https://doaj.org/article/0a0a0c0eeb834eddadd29d0c1f98b256
Autor:
Chiara Soldati, Irene Lopez‐Fabuel, Luca G Wanderlingh, Marina Garcia‐Macia, Jlenia Monfregola, Alessandra Esposito, Gennaro Napolitano, Marta Guevara‐Ferrer, Anna Scotto Rosato, Einar K Krogsaeter, Dominik Paquet, Christian M Grimm, Sandro Montefusco, Thomas Braulke, Stephan Storch, Sara E Mole, Maria A De Matteis, Andrea Ballabio, Julio L Sampaio, Tristan McKay, Ludger Johannes, Juan P Bolaños, Diego L Medina
Publikováno v:
EMBO Molecular Medicine, Vol 13, Iss 10, Pp 1-19 (2021)
Abstract Batten diseases (BDs) are a group of lysosomal storage disorders characterized by seizure, visual loss, and cognitive and motor deterioration. We discovered increased levels of globotriaosylceramide (Gb3) in cellular and murine models of CLN
Externí odkaz:
https://doaj.org/article/d6c8d9dd375c49b29d6deee58e6d431f
Publikováno v:
EMBO Molecular Medicine, Vol 13, Iss 2, Pp 1-21 (2021)
Abstract Lysosomal storage diseases are a group of metabolic disorders caused by deficiencies of several components of lysosomal function. Most commonly affected are lysosomal hydrolases, which are involved in the breakdown and recycling of a variety
Externí odkaz:
https://doaj.org/article/af55b965be18440e8fdcf0608db34707
Autor:
Francesco Agostini, Rossella Agostinis, Diego L. Medina, Marco Bisaglia, Elisa Greggio, Nicoletta Plotegher
Publikováno v:
Molecular Neurobiology. 59:5000-5023
The microphthalmia/transcription factor E (MiTF/TFE) transcription factors are responsible for the regulation of various key processes for the maintenance of brain function, including autophagy-lysosomal pathway, lipid catabolism, and mitochondrial h
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4791300bcdba799cc437b7d594f0648b
https://doi.org/10.1007/s12035-022-02895-3
https://doi.org/10.1007/s12035-022-02895-3
Autor:
Elena Marchesan, Alice Nardin, Sofia Mauri, Simone Di Paola, Monica Chinellato, Sophia von Stockum, Joy Chakraborty, Stephanie Herkenne, Valentina Basso, Emilie Schrepfer, Oriano Marin, Laura Cendron, Diego L. Medina, Luca Scorrano, Elena Ziviani
Selective removal of dysfunctional mitochondria via autophagy is crucial for the maintenance of cellular homeostasis. This event is initiated by the translocation of the E3 ubiquitin ligase Parkin to damaged mitochondria, and it requires the Serine/T
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::1d59807936b137877ca22580545900f8
https://doi.org/10.1101/2023.01.31.526442
https://doi.org/10.1101/2023.01.31.526442
Autor:
Anna Scotto Rosato, Einar K Krogsaeter, Dawid Jaślan, Carla Abrahamian, Sandro Montefusco, Chiara Soldati, Barbara Spix, Maria Teresa Pizzo, Giuseppina Grieco, Julia Böck, Amanda Wyatt, Daniela Wünkhaus, Marcel Passon, Marc Stieglitz, Marco Keller, Guido Hermey, Sandra Markmann, Doris Gruber‐Schoffnegger, Susan Cotman, Ludger Johannes, Dennis Crusius, Ulrich Boehm, Christian Wahl‐Schott, Martin Biel, Franz Bracher, Elvira De Leonibus, Elena Polishchuk, Diego L Medina, Dominik Paquet, Christian Grimm
Publikováno v:
EMBO Molecular Medicine. 14
Lysosomes are cell organelles that degrade macromolecules to recycle their components. If lysosomal degradative function is impaired, e.g., due to mutations in lysosomal enzymes or membrane proteins, lysosomal storage diseases (LSDs) can develop. LSD
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e0beaf2f72a21d6c00110b832eac5e3f
https://doi.org/10.15252/emmm.202115377
https://doi.org/10.15252/emmm.202115377