C
Cristina Ugalde
Researcher at Carlos III Health Institute
Publications - 58
Citations - 3209
Cristina Ugalde is an academic researcher from Carlos III Health Institute. The author has contributed to research in topics: Mitochondrion & Respiratory chain. The author has an hindex of 29, co-authored 52 publications receiving 2822 citations. Previous affiliations of Cristina Ugalde include Radboud University Nijmegen Medical Centre & Radboud University Nijmegen.
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Journal ArticleDOI
Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency
Cristina Ugalde,Rolf J.R.J. Janssen,Lambert P. van den Heuvel,Jan A.M. Smeitink,Leo G.J. Nijtmans +4 more
TL;DR: Blue native electrophoresis is used in order to study how different nuclear mutations affect the integrity of mitochondrial OXPHOS complexes in fibroblasts from 15 complex I-deficient patients to point more selectively to candidate genes for pathogenic mutations that could lead to an isolated complex I defect.
Journal ArticleDOI
Mitochondrial complex I plays an essential role in human respirasome assembly.
David Moreno-Lastres,Flavia Fontanesi,Inés García-Consuegra,Miguel A. Martín,Joaquín Arenas,Antoni Barrientos,Cristina Ugalde +6 more
TL;DR: It is shown that in human cells, respirasome biogenesis involves a complex I assembly intermediate acting as a scaffold for the combined incorporation of complexes III and IV subunits, rather than originating from the association of preassembled individual holoenzymes.
Journal ArticleDOI
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies
Cristina Ugalde,Rutger O. Vogel,Richard Huijbens,Bert van den Heuvel,Jan A.M. Smeitink,Leo G.J. Nijtmans +5 more
TL;DR: The results suggest that human complex I assembly resembles that of Neurospora crassa, in which a membrane arm is formed and assembled to a preformed peripheral arm, and support ideas about modular evolution.
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Mutant mitochondrial elongation factor G1 and combined oxidative phosphorylation deficiency
Marieke J H Coenen,Hana Antonicka,Cristina Ugalde,Florin Sasarman,Rainer Rossi,J.G.A.M. Angelien Heister,Robert F. Newbold,Frans J.M. Trijbels,Lambert P. van den Heuvel,Eric A. Shoubridge,Jan A.M. Smeitink +10 more
TL;DR: Two siblings with a severe defect in mitochondrial translation, reduced levels of oxidative phosphorylation complexes containing mitochondrial DNA (mtDNA)-encoded subunits, and progressive hepatoencephalopathy are investigated, defining a new class of gene defects underlying disorders of oxidativeosphorylation.
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Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.
Rosa Pello,Miguel A. Martín,Valerio Carelli,Leo G.J. Nijtmans,Alessandro Achilli,Alessandro Achilli,Maria Pala,Antonio Torroni,Aurora Gomez-Duran,Eduardo Ruiz-Pesini,Andrea Martinuzzi,Jan A.M. Smeitink,Joaquín Arenas,Cristina Ugalde +13 more
TL;DR: The results showed differentially delayed assembly rates of respiratory chain complexes I, III and IV amongst mutants belonging to different mtDNA haplogroups, revealing that specific mtDNA polymorphisms may modify the pathogenic potential of LHON mutations by affecting the overall assembly kinetics of OXPHOS complexes.