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Clemente Perez, Paula paula.clemente@uam.es

Bioquímica / Universidad Autónoma de Madrid

Publications

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
  • Rackham O
  • Filipovska A
  • Rorbach J
  • Freyer C
  • Wredenberg A
... View more Collapse

Nature Communications - 1/12/2022

10.1038/s41467-022-33368-9 View at source

  • ISSN 20411723

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
  • Chernogubova E
  • Begzati A
  • Wibom R
  • Jain M
  • Nilsson R
  • Käll L
  • Wedell A
  • Freyer C
  • Wredenberg A
... View more Collapse

Science Advances - 19/2/2021

10.1126/sciadv.abf0717 View at source

  • ISSN 23752548

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
  • Wredenberg A
... View more Collapse

Plos Genetics - 1/7/2019

10.1371/journal.pgen.1008240 View at source

  • ISSN 15537390

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
  • Wedell A
... View more Collapse

Molecular Genetics & Genomic Medicine - 1/6/2019

10.1002/mgg3.654 View at source

  • ISSN 23249269

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
  • Freyer C
  • Wredenberg A
... View more Collapse

Stem Cell Reports (p. 696-711) - 9/4/2019

10.1016/j.stemcr.2019.01.023 View at source

  • ISSN 22136711

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
  • Obinger, Christian
  • Battistuzzi, Gianantonio
  • Bellei, Marzia
  • Borsari, Marco
  • Di Rocco, Giulia
  • Viola, Helena M.
  • Hoo, Livia C.
  • Cladera, Josep
  • Lagerstedt-Robinson, Kristina
  • Xiang, Fengqing
  • Wredenberg, Anna
  • Miralles, Francesc
  • Jose Baiges, Juan
  • Malfatti, Edoardo
  • Romero, Norma B.
  • Streichenberger, Nathalie
  • Via, 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, Noemi
  • Ferrer, Isidre
  • Edstrom, Lars
  • Wedell, Anna
  • Laing, Nigel G.;
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Nature Communications - 1/12/2019

10.1038/s41467-019-09111-2

  • ISSN 20411723
  • iMarina

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
... View more Collapse

Science Advances - 1/1/2019

10.1126/sciadv.aav9824 View at source

  • ISSN 23752548

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
  • Peter B
  • Freyer C
  • Falkenberg M
  • Wredenberg A
... View more Collapse

Human Molecular Genetics (p. 2515-2525) - 1/7/2017

10.1093/hmg/ddx146 View at source

  • ISSN 14602083

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
... View more Collapse

Plos Genetics - 1/5/2016

10.1371/journal.pgen.1006028 View at source

  • ISSN 15537390

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
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Biochemistry (p. 6209-6220) - 15/11/2016

10.1021/acs.biochem.6b00644 View at source

  • ISSN 00062960

This researcher has no books.

Mitochondrial DNA replication in health and disease

  • Gallardo, María Esther
  • Clemente Pérez, Paula
  • Echevarría Zamora, Lucía
  • Peralta, Susana
  • Garesse, Rafael

Mitochondrial Pathophysiology (p. 115-143) - 1/1/2011

  • iMarina

This researcher has no conferences.

This researcher has no working papers.

This researcher has no technical reports.

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))
... View more Collapse

Period: 01-01-2011 - 31-12-2013

  • iMarina

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))
... View more Collapse

Period: 01-01-2008 - 31-12-2010

  • iMarina

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

20/4/2012

  • iMarina

This researcher has no patents or software licenses.

Last data update: 3/18/24 11:33 AM