Zobrazeno 1 - 10
of 129
pro vyhledávání: '"Erika Fernandez"'
Autor:
Irene H. Flønes, Lilah Toker, Dagny Ann Sandnes, Martina Castelli, Sepideh Mostafavi, Njål Lura, Omnia Shadad, Erika Fernandez-Vizarra, Cèlia Painous, Alexandra Pérez-Soriano, Yaroslau Compta, Laura Molina-Porcel, Guido Alves, Ole-Bjørn Tysnes, Christian Dölle, Gonzalo S. Nido, Charalampos Tzoulis
Publikováno v:
Nature Communications, Vol 15, Iss 1, Pp 1-18 (2024)
Abstract Idiopathic Parkinson’s disease (iPD) is believed to have a heterogeneous pathophysiology, but molecular disease subtypes have not been identified. Here, we show that iPD can be stratified according to the severity of neuronal respiratory c
Externí odkaz:
https://doaj.org/article/ca1314f7e4514676b9bb0eefc96db530
Autor:
Dora Ravasz, David Bui, Sara Nazarian, Gergely Pallag, Noemi Karnok, Jennie Roberts, Bryan P. Marzullo, Daniel A. Tennant, Bennett Greenwood, Alex Kitayev, Collin Hill, Timea Komlódi, Carolina Doerrier, Kristyna Cunatova, Erika Fernandez-Vizarra, Erich Gnaiger, Michael A. Kiebish, Alexandra Raska, Krasimir Kolev, Bence Czumbel, Niven R. Narain, Thomas N. Seyfried, Christos Chinopoulos
Publikováno v:
Scientific Reports, Vol 14, Iss 1, Pp 1-21 (2024)
Abstract Anoxia halts oxidative phosphorylation (OXPHOS) causing an accumulation of reduced compounds in the mitochondrial matrix which impedes dehydrogenases. By simultaneously measuring oxygen concentration, NADH autofluorescence, mitochondrial mem
Externí odkaz:
https://doaj.org/article/453486a76e924fb8b9b5af5d51ebc216
Publikováno v:
STAR Protocols, Vol 3, Iss 2, Pp 101322- (2022)
Summary: Mitochondrial respiratory chain (MRC) dysfunction is linked to mitochondrial disease as well as other common conditions such as diabetes, neurodegeneration, cancer, and aging. Thus, the evaluation of MRC enzymatic activities is fundamental f
Externí odkaz:
https://doaj.org/article/311f33c604a049cb98053f37ec51f6de
Publikováno v:
Biomolecules, Vol 13, Iss 2, p 378 (2023)
The fruit fly—i.e., Drosophila melanogaster—has proven to be a very useful model for the understanding of basic physiological processes, such as development or ageing. The availability of straightforward genetic tools that can be used to produce
Externí odkaz:
https://doaj.org/article/ab017d7e81654afba8decb80271f401e
Autor:
Luigi D’Angelo, Elisa Astro, Monica De Luise, Ivana Kurelac, Nikkitha Umesh-Ganesh, Shujing Ding, Ian M. Fearnley, Giuseppe Gasparre, Massimo Zeviani, Anna Maria Porcelli, Erika Fernandez-Vizarra, Luisa Iommarini
Publikováno v:
Cell Reports, Vol 35, Iss 3, Pp 109002- (2021)
Summary: Complex I (CI) is the largest enzyme of the mitochondrial respiratory chain, and its defects are the main cause of mitochondrial disease. To understand the mechanisms regulating the extremely intricate biogenesis of this fundamental bioenerg
Externí odkaz:
https://doaj.org/article/fff9d8e4d29741f482d85734394b1c78
Autor:
Luca Peruzzotti-Jametti, Joshua D Bernstock, Cory M Willis, Giulia Manferrari, Rebecca Rogall, Erika Fernandez-Vizarra, James C Williamson, Alice Braga, Aletta van den Bosch, Tommaso Leonardi, Grzegorz Krzak, Ágnes Kittel, Cristiane Benincá, Nunzio Vicario, Sisareuth Tan, Carlos Bastos, Iacopo Bicci, Nunzio Iraci, Jayden A Smith, Ben Peacock, Karin H Muller, Paul J Lehner, Edit Iren Buzas, Nuno Faria, Massimo Zeviani, Christian Frezza, Alain Brisson, Nicholas J Matheson, Carlo Viscomi, Stefano Pluchino
Publikováno v:
PLoS Biology, Vol 19, Iss 4, p e3001166 (2021)
Neural stem cell (NSC) transplantation induces recovery in animal models of central nervous system (CNS) diseases. Although the replacement of lost endogenous cells was originally proposed as the primary healing mechanism of NSC grafts, it is now cle
Externí odkaz:
https://doaj.org/article/62e32062ab904101a16fc0e8ef20d45c
Autor:
Simonetta Andreazza, Colby L. Samstag, Alvaro Sanchez-Martinez, Erika Fernandez-Vizarra, Aurora Gomez-Duran, Juliette J. Lee, Roberta Tufi, Michael J. Hipp, Elizabeth K. Schmidt, Thomas J. Nicholls, Payam A. Gammage, Patrick F. Chinnery, Michal Minczuk, Leo J. Pallanck, Scott R. Kennedy, Alexander J. Whitworth
Publikováno v:
Nature Communications, Vol 10, Iss 1, Pp 1-14 (2019)
The role of mitochondrial DNA mutations in organismal fitness and lifespan have been studied in mitochondrial mutator models with varying results. Here, the authors generate a new APOBEC1 expression-based Drosophila mutator model and show that it has
Externí odkaz:
https://doaj.org/article/f090eaf1785247208a099d9f6a996bb3
Autor:
Michela Di Nottia, Maria Marchese, Daniela Verrigni, Christian Daniel Mutti, Alessandra Torraco, Romina Oliva, Erika Fernandez-Vizarra, Federica Morani, Giulia Trani, Teresa Rizza, Daniele Ghezzi, Anna Ardissone, Claudia Nesti, Gessica Vasco, Massimo Zeviani, Michal Minczuk, Enrico Bertini, Filippo Maria Santorelli, Rosalba Carrozzo
Publikováno v:
Neurobiology of Disease, Vol 141, Iss , Pp 104880- (2020)
Mitochondrial ribosomal protein large 24 (MRPL24) is 1 of the 82 protein components of mitochondrial ribosomes, playing an essential role in the mitochondrial translation process.We report here on a baby girl with cerebellar atrophy, choreoathetosis
Externí odkaz:
https://doaj.org/article/fd38a99915094d3995c8278007993be3
Autor:
Sara Vidoni, Michael E. Harbour, Sergio Guerrero-Castillo, Alba Signes, Shujing Ding, Ian M. Fearnley, Robert W. Taylor, Valeria Tiranti, Susanne Arnold, Erika Fernandez-Vizarra, Massimo Zeviani
Publikováno v:
Cell Reports, Vol 18, Iss 7, Pp 1727-1738 (2017)
The biogenesis of human cytochrome c oxidase (COX) is an intricate process in which three mitochondrial DNA (mtDNA)-encoded core subunits are assembled in a coordinated way with at least 11 nucleus-encoded subunits. Many chaperones shared between yea
Externí odkaz:
https://doaj.org/article/d2f36c1008504deab7320891faca21e8
Autor:
Michele Brischigliaro, Samantha Corrà, Claudia Tregnago, Erika Fernandez-Vizarra, Massimo Zeviani, Rodolfo Costa, Cristiano De Pittà
Publikováno v:
Frontiers in Physiology, Vol 10 (2019)
Cytochrome c oxidase (COX) deficiency is the biochemical hallmark of several mitochondrial disorders, including subjects affected by mutations in apoptogenic-1 (APOPT1), recently renamed as COA8 (HGNC:20492). Loss-of-function mutations are responsibl
Externí odkaz:
https://doaj.org/article/434ff7e64d5f492bbc89a701f9cfeec9