C
Christopher A. Powell
Researcher at University of Cambridge
Publications - 37
Citations - 2126
Christopher A. Powell is an academic researcher from University of Cambridge. The author has contributed to research in topics: Mitochondrial DNA & Mitochondrion. The author has an hindex of 21, co-authored 33 publications receiving 1574 citations. Previous affiliations of Christopher A. Powell include Medical Research Council & MRC Mitochondrial Biology Unit.
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Journal ArticleDOI
PrimPol, an Archaic Primase/Polymerase Operating in Human Cells
Sara García-Gómez,Aurelio Reyes,María I. Martínez-Jiménez,E. Sandra Chocron,Silvana Mouron,Gloria Terrados,Christopher A. Powell,Eduardo Salido,Juan Méndez,Ian J. Holt,Luis Blanco +10 more
TL;DR: A second primase in human cells, PrimPol, is described, which has the ability to start DNA chains with deoxynucleotides unlike regular primases, which use exclusively ribonucleotide, which is a DNA polymerase tailored to bypass the most common oxidative lesions in DNA.
Journal ArticleDOI
Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo.
Payam A. Gammage,Carlo Viscomi,Marie-Lune Simard,Ana S. H. Costa,Edoardo Gaude,Christopher A. Powell,Lindsey Van Haute,Beverly J. McCann,Pedro Rebelo-Guiomar,Pedro Rebelo-Guiomar,Raffaele Cerutti,Lei Zhang,Edward J. Rebar,Massimo Zeviani,Christian Frezza,James B. Stewart,Michal Minczuk +16 more
TL;DR: Findings constitute proof of principle that mtDNA heteroplasmy correction using programmable nucleases could provide a therapeutic route for heteroplasmic mitochondrial diseases of diverse genetic origin.
Journal ArticleDOI
Deficient methylation and formylation of mt-tRNA Met wobble cytosine in a patient carrying mutations in NSUN3
Lindsey Van Haute,Sabine Dietmann,Laura S. Kremer,Shobbir Hussain,Sarah F. Pearce,Christopher A. Powell,Joanna Rorbach,Rebecca Lantaff,Sandra Blanco,Sascha Sauer,Sascha Sauer,Sascha Sauer,Urania Kotzaeridou,Georg F. Hoffmann,Yasin Memari,Anja Kolb-Kokocinski,Richard Durbin,Johannes A. Mayr,Michaela Frye,Holger Prokisch,Michal Minczuk +20 more
TL;DR: It is shown that NSun3 is required for deposition of m5C at the anticodon loop in the mitochondrially encoded transfer RNA methionine (mt-tRNAMet), and f5C in human mitochondrial RNA is generated by oxidative processing of m 5C.
Journal ArticleDOI
Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances
Sarah F. Pearce,Pedro Rebelo-Guiomar,Pedro Rebelo-Guiomar,Aaron R. D’Souza,Christopher A. Powell,Lindsey Van Haute,Michal Minczuk +6 more
TL;DR: The latest breakthroughs in understanding of the post-transcriptional processes of mitochondrial gene expression are reviewed, focusing on advances in analyzing the mitochondrial epitranscriptome, the role of mitochondrial RNA granules (MRGs), the benefits of recently obtained structures of the mitochondrial ribosome, and the coordination of mitochondrial and cytosolic translation to orchestrate the biogenesis of OxPhos complexes.
Journal ArticleDOI
Mutations in GTPBP3 Cause a Mitochondrial Translation Defect Associated with Hypertrophic Cardiomyopathy, Lactic Acidosis, and Encephalopathy
Robert Kopajtich,Thomas J. Nicholls,Joanna Rorbach,Metodi D. Metodiev,Peter Freisinger,Hanna Mandel,Arnaud Vanlander,Daniele Ghezzi,Rosalba Carrozzo,Robert W. Taylor,Klaus Marquard,Kei Murayama,Thomas Wieland,Thomas Schwarzmayr,Johannes A. Mayr,Sarah F. Pearce,Christopher A. Powell,Ann Saada,Akira Ohtake,Federica Invernizzi,Eleonora Lamantea,Ewen W. Sommerville,Angela Pyle,Patrick F. Chinnery,Ellen Crushell,Yasushi Okazaki,Masakazu Kohda,Yoshihito Kishita,Yoshimi Tokuzawa,Zahra Assouline,Marlène Rio,François Feillet,Bénédict Mousson de Camaret,Dominique Chretien,Arnold Munnich,Arnold Munnich,Björn Menten,Tom Sante,Joél Smet,Luc Régal,Abraham Lorber,Asaad Khoury,Massimo Zeviani,Tim M. Strom,Thomas Meitinger,Enrico Bertini,Rudy Van Coster,Thomas Klopstock,Agnès Rötig,Tobias B. Haack,Michal Minczuk,Holger Prokisch +51 more
TL;DR: The study of a mitochondrial translation disorder points toward the importance of posttranscriptional modification of mitochondrial tRNAs for proper mitochondrial function.