Human COA3 Is an Oligomeric Highly Flexible Protein in Solution - Neira JL; Martínez-Rodríguez S; Hernández-Cifre JG; Cámara-Artigas A; Clemente P; Peralta S; Fernández-Moreno MÁ; Garesse R; García De La Torre J; Rizzuti B (Biochemistry, 2016)

Glutamyl-tRNAGln amidotransferase is essential for mammalian mitochondrial translation in vivo - Echevarría L; Clemente P; Her?andez-Sierra R; Gallardo M; Fer?andez-Moreno M; Garesse R (Biochemical Journal, 2014)

Evaluation of mitochondrial function and metabolic reprogramming during tumor progression in a cell model of skin carcinogenesis - Vallejo C., Cruz-Bermúdez A., Clemente P., Hernández-Sierra R., Garesse R., Quintanilla M. (Biochimie, 2013)

HCOA3 stabilizes cytochrome c oxidase 1 (COX1) and promotes cytochrome c oxidase assembly in human mitochondria - Clemente P; Peralta S; Cruz-Bermudez A; Echevarría L; Fontanesi F; Barrientos A; Fernandez-Moreno M; Garesse R (Journal Of Biological Chemistry, 2013)

Coiled coil domain-containing protein 56 (CCDC56) is a novel mitochondrial protein essential for cytochrome c oxidase function - Peralta S; Clemente P; Sánchez-Martínez A; Calleja M; Hernández-Sierra R; Matsushima Y; Adán C; Ugalde C; Fernández-Moreno MA; Kaguni LS; Garesse R (Journal Of Biological Chemistry, 2012)

CCDC56, una proteína esencial para la formación de la citocromo c oxidasa en células humanas - Clemente Perez, Paula (Autor o Coautor); Garesse Alarcon, Rafael (Director); Fernandez Moreno, Miguel Angel (Director); Fernandez Piqueras, Jose (Autor o Coautor) Doctorando: CLEMENTE PEREZ, PAULA (2012)

Caracterización funcional de defectos del sistema de fosforilación oxidativa causantes de enfermedades mitocondriales. - Maria Carmen Prior de Castro (Investigador/a); Rosana Hernandez Sierra (Investigador/a); Clemente Perez, Paula (Investigador/a); Fernandez Moreno, Miguel Angel (Investigador/a); Gallardo Perez, Maria Esther (Investigador/a); Carmen Prior Castro (Investigador/a); Carmen Garcia Vallejo (Investigador/a); Garesse Alarcon, Rafael (Investigador principal (IP)) (2011)

Mitochondrial DNA replication in health and disease - Gallardo, María Esther; Clemente Pérez, Paula; Echevarría Zamora, Lucía; Peralta, Susana; Garesse, Rafael (Mitochondrial Pathophysiology, 2011)

Acción coordinada para el estudio de los mecanismos determinantes de la expresión fenotípica de mutaciones en genes reguladores del sistema de fosforilación oxidativa (parte 3: variabilidad genotípica del MTDNA y defectos de. - González Vioque, Emiliano (Investigador/a); Sánchez Martínez, Álvaro (Investigador/a); Echevarria Zamora, Lucia (Investigador/a); Clemente Perez, Paula (Investigador/a); Veronica Domingo Rodriguez (Investigador/a); Rosana Hernandez Sierra (Investigador/a); Fernandez Moreno, Miguel Angel (Investigador/a); Gallardo Perez, Maria Esther (Investigador/a); Isabel Sanjuan Bilbao (Investigador/a); Carmen Garcia Vallejo (Investigador/a); Garesse Alarcon, Rafael (Investigador principal (IP)) (2008)

Modeling human mitochondrial diseases in flies - Sánchez-Martínez A; Luo N; Clemente P; Adán C; Hernández-Sierra R; Ochoa P; Fernández-Moreno M; Kaguni L; Garesse R (Biochimica Et Biophysica Acta-Bioenergetics, 2006)

ANGEL2 phosphatase activity is required for non-canonical mitochondrial RNA processing - Clemente P; Calvo-Garrido J; Pearce SF; Schober FA; Shigematsu M; Siira SJ; Laine I; Spåhr H; Steinmetzger C; Petzold K; Kirino Y; Wibom R; et al. (Nature Communications, 2022)

The one-carbon pool controls mitochondrial energy metabolism via complex i and iron-sulfur clusters - Schober FA; Moore D; Atanassov I; Moedas MF; Clemente P; Végvári Á; Fissi NE; Filograna R; Bucher AL; Hinze Y; The M; Hedman E; et al. (Science Advances, 2021)

Myoglobinopathy is an adult-onset autosomal dominant myopathy with characteristic sarcoplasmic inclusions - Olive, 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, Nuria; Hofbauer, Stefan; et al. (Nature Communications, 2019)

Defects of mitochondrial RNA turnover lead to the accumulation of double-stranded RNA in vivo - Pajak A; Laine I; Clemente P; El-Fissi N; Schober FA; Maffezzini C; Calvo-Garrido J; Wibom R; Filograna R; Dhir A; Wedell A; Freyer C; et al. (Plos Genetics, 2019)

Mutations in the mitochondrial tryptophanyl-tRNA synthetase cause growth retardation and progressive leukoencephalopathy - Maffezzini C; Laine I; Dallabona C; Clemente P; Calvo-Garrido J; Wibom R; Naess K; Barbaro M; Falk A; Donnini C; Freyer C; Wredenberg A; et al. (Molecular Genetics & Genomic Medicine, 2019)

SQSTM1/p62-Directed Metabolic Reprogramming Is Essential for Normal Neurodifferentiation - Calvo-Garrido J; Maffezzini C; Schober FA; Clemente P; Uhlin E; Kele M; Stranneheim H; Lesko N; Bruhn H; Svenningsson P; Falk A; Wedell A; et al. (Stem Cell Reports, 2019)

Modulation of mtDNA copy number ameliorates the pathological consequences of a heteroplasmic mtDNA mutation in the mouse - Filograna R; Koolmeister C; Upadhyay M; Pajak A; Clemente P; Wibom R; Simard ML; Wredenberg A; Freyer C; Stewart JB; Larsson NG (Science Advances, 2019)

A multi-systemic mitochondrial disorder due to a dominant p. Y955H disease variant in DNA polymerase gamma - Siibak T; Clemente P; Bratic A; Bruhn H; Kauppila T; Macao B; Schober F; Lesko N; Wibom R; Naess K; Nennesmo I; Wedell A; et al. (Human Molecular Genetics, 2017)

Mitochondrial Polyadenylation Is a One-Step Process Required for mRNA Integrity and tRNA Maturation - Bratic A; Clemente P; Calvo-Garrido J; Maffezzini C; Felser A; Wibom R; Wedell A; Freyer C; Wredenberg A (Plos Genetics, 2016)

SUV3 helicase is required for correct processing of mitochondrial transcripts - Clemente P; Pajak A; Laine I; Wibom R; Wedell A; Freyer C; Wredenberg A (Nucleic Acids Research, 2015)

Cox25 teams up with Mss51, Ssc1, and Cox14 to regulate mitochondrial cytochrome c oxidase subunit 1 expression and assembly in Saccharomyces cerevisiae - Fontanesi F; Clemente P; Barrientos A (Journal Of Biological Chemistry, 2011)