SUV3 helicase is required for correct processing of mitochondrial transcripts

por Clemente, Paula, Pajak, Aleksandra, Laine, Isabelle, Wibom, Rolf, Wedell, Anna, Freyer, Christoph, Wredenberg, Anna
Publicado 2015

Mitochondrial Polyadenylation Is a One-Step Process Required for mRNA Integrity and tRNA Maturation

por Bratic, Ana, Clemente, Paula, Calvo-Garrido, Javier, Maffezzini, Camilla, Felser, Andrea, Wibom, Rolf, Wedell, Anna, Freyer, Christoph, Wredenberg, Anna
Publicado 2016

Mutations in the mitochondrial tryptophanyl‐tRNA synthetase cause growth retardation and progressive leukoencephalopathy

por Maffezzini, Camilla, Laine, Isabelle, Dallabona, Cristina, Clemente, Paula, Calvo‐Garrido, Javier, Wibom, Rolf, Naess, Karin, Barbaro, Michela, Falk, Anna, Donnini, Claudia, Freyer, Christoph, Wredenberg, Anna, Wedell, Anna
Publicado 2019

Defects of mitochondrial RNA turnover lead to the accumulation of double-stranded RNA in vivo

por Pajak, Aleksandra, Laine, Isabelle, Clemente, Paula, El-Fissi, Najla, Schober, Florian A., Maffezzini, Camilla, Calvo-Garrido, Javier, Wibom, Rolf, Filograna, Roberta, Dhir, Ashish, Wedell, Anna, Freyer, Christoph, Wredenberg, Anna
Publicado 2019

SQSTM1/p62-Directed Metabolic Reprogramming Is Essential for Normal Neurodifferentiation

por Calvo-Garrido, Javier, Maffezzini, Camilla, Schober, Florian A., Clemente, Paula, Uhlin, Elias, Kele, Malin, Stranneheim, Henrik, Lesko, Nicole, Bruhn, Helene, Svenningsson, Per, Falk, Anna, Wedell, Anna, Freyer, Christoph, Wredenberg, Anna
Publicado 2019

A multi-systemic mitochondrial disorder due to a dominant p.Y955H disease variant in DNA polymerase gamma

por Siibak, Triinu, Clemente, Paula, Bratic, Ana, Bruhn, Helene, Kauppila, Timo E.S., Macao, Bertil, Rosenberger, Florian A., Lesko, Nicole, Wibom, Rolf, Naess, Karin, Nennesmo, Inger, Wedell, Anna, Peter, Bradley, Freyer, Christoph, Falkenberg, Maria, Wredenberg, Anna
Publicado 2017

ANGEL2 phosphatase activity is required for non-canonical mitochondrial RNA processing

por Clemente, Paula, Calvo-Garrido, Javier, Pearce, Sarah F., Schober, Florian A., Shigematsu, Megumi, Siira, Stefan J., Laine, Isabelle, Spåhr, Henrik, Steinmetzger, Christian, Petzold, Katja, Kirino, Yohei, Wibom, Rolf, Rackham, Oliver, Filipovska, Aleksandra, Rorbach, Joanna, Freyer, Christoph, Wredenberg, Anna
Publicado 2022

The one-carbon pool controls mitochondrial energy metabolism via complex I and iron-sulfur clusters

por Schober, Florian A., Moore, David, Atanassov, Ilian, Moedas, Marco F., Clemente, Paula, Végvári, Ákos, Fissi, Najla El, Filograna, Roberta, Bucher, Anna-Lena, Hinze, Yvonne, The, Matthew, Hedman, Erik, Chernogubova, Ekaterina, Begzati, Arjana, Wibom, Rolf, Jain, Mohit, Nilsson, Roland, Käll, Lukas, Wedell, Anna, Freyer, Christoph, Wredenberg, Anna
Publicado 2021

Myoglobinopathy is an adult-onset autosomal dominant myopathy with characteristic sarcoplasmic inclusions

por Olivé, Montse, Engvall, Martin, Ravenscroft, Gianina, Cabrera-Serrano, Macarena, Jiao, Hong, Bortolotti, Carlo Augusto, Pignataro, Marcello, Lambrughi, Matteo, Jiang, Haibo, Forrest, Alistair R. R., Benseny-Cases, Núria, Hofbauer, Stefan, Obinger, Christian, Battistuzzi, Gianantonio, Bellei, Marzia, Borsari, Marco, Di Rocco, Giulia, Viola, Helena M., Hool, Livia C., Cladera, Josep, Lagerstedt-Robinson, Kristina, Xiang, Fengqing, Wredenberg, Anna, Miralles, Francesc, Baiges, Juan José, Malfatti, Edoardo, Romero, Norma B., Streichenberger, Nathalie, Vial, Christophe, Claeys, Kristl G., Straathof, Chiara S. M., Goris, An, Freyer, Christoph, Lammens, Martin, Bassez, Guillaume, Kere, Juha, Clemente, Paula, Sejersen, Thomas, Udd, Bjarne, Vidal, Noemí, Ferrer, Isidre, Edström, Lars, Wedell, Anna, Laing, Nigel G.
Publicado 2019