Publications | Centro de Investigaciones Biológicas Margarita Salas

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Liang Chao, Padavannil Abhilash, Zhang Shan, Beh Sheryl, Robinson David R.L., Meisterknecht Jana, Cabrera-Orefice Alfredo, Koves Timothy R., Watanabe Chika, Watanabe Miyuki, Illescas María, Lim Radiance, Johnson Jordan M., Ren Shuxun, Wu Ya-Jun, Kappei Dennis, Ghelli Anna Maria, Funai Katsuhiko, Osaka Hitoshi, Muoio Deborah, Ugalde Cristina, Wittig Ilka, Stroud David A., Letts James A., Ho Lena [2025]. Formation of I2+III2 supercomplex rescues respiratory chain defects. Cell Metab . 2025 Jan 8:S1550-4131(24)00457-1. doi: 10.1016/j.cmet.2024.11.011.

Lisowski, Pawel, Lickfett, Selene, Rybak-Wolf, Agnieszka, Menacho, Carmen, Le, Stephanie, Pentimalli, Tancredi Massimo, Notopoulou, Sofia, Dykstra, Werner, Oehler, Daniel, López-Calcerrada, Sandra, Mlody, Barbara, Otto, Maximilian, Wu, Haijia, Richter, Yasmin, Roth, Philipp, Anand, Ruchika, Kulka, Linda A. M., Meierhofer, David, Glazar, Petar, Legnini, Ivano, Telugu, Narasimha Swamy, Hahn, Tobias, Neuendorf, Nancy, Miller, Duncan C., Böddrich, Annett, Polzin, Amin, Mayatepek, Ertan, Diecke, Sebastian, Olzscha, Heidi, Kirstein, Janine, Ugalde, Cristina, Petrakis, Spyros, Cambridge, Sidney, Rajewsky, Nikolaus, Kühn, Ralf, Wanker, Erich E., Priller, Josef, Metzger, Jakob J., Prigione, Alessandro [2024]. Mutant huntingtin impairs neurodevelopment in human brain organoids through CHCHD2-mediated neurometabolic failure. Nat Commun. 2024 Aug 22;15(1):7027.

Baden, Pascale, Perez, Maria Jose, Raji, Hariam, Bertoli, Federico, Kalb, Stefanie, Illescas, María, Spanos, Fokion, Giuliano, Claudio, Calogero, Alessandra Maria, Oldrati, Marvin, Hebestreit, Hannah, Cappelletti, Graziella, Brockmann, Kathrin, Gasser, Thomas, Schapira, Anthony H. V., Ugalde, Cristina, Deleidi, Michela [2023]. Glucocerebrosidase is imported into mitochondria and preserves complex I integrity and energy metabolism. Nat Commun. 2023 Apr 6;14(1):1930.

Aye, Cho-Cho , Hammond, Dean E. , Rodriguez-Cuenca, Sergio , Doherty, Mary K. , Whitfield, Phillip D. , Phelan, Marie M. , Yang, Chenjing , Perez-Perez, Rafael , Li, Xiaoxin , Diaz-Ramos, Angels , Peddinti, Gopal , Oresic, Matej , Vidal-Puig, Antonio , Zorzano, Antonio , Ugalde, Cristina , Mora, Silvia [2023]. CBL/CAP Is Essential for Mitochondria Respiration Complex I Assembly and Bioenergetics Efficiency in Muscle Cells. Int J Mol Sci. 2023 Feb 8;24(4):3399.

Amate-García, Guillermo , Ballesta-Martínez, María J. , Serrano-Lorenzo, Pablo , Garrido-Moraga, Rocío , González-Quintana, Adrián , Blázquez, Alberto , Rubio, Juan C. , García-Consuegra, Inés , Arenas, Joaquín , Ugalde, Cristina , Morán, María , Guillén-Navarro, Encarnación , Martín, Miguel A. [2023]. A Novel Mutation Associated with Neonatal Lethal Cardiomyopathy Leads to an Alternative Transcript Expression in the X-Linked Complex I NDUFB11 Gene. Int J Mol Sci. 2023 Jan 16;24(2):1743.

Fernández-Vizarra, Erika, Ugalde, Cristina [2022]. Cooperative assembly of the mitochondrial respiratory chain. Trends Biochem Sci. 2022 Dec;47(12):999-1008.

Fernández-Vizarra, Erika, López-Calcerrada, Sandra, Sierra-Magro, Ana, Pérez-Pérez, Rafael, Formosa, Luke E., Hock, Daniella H., Illescas, María, Peñas, Ana, Brischigliaro, Michele, Ding, Shujing, Fearnley, Ian M., Tzoulis, Charalampos, Pitceathly, Robert D.S., Arenas, Joaquín, Martín, Miguel A., Stroud, David A., Zeviani, Massimo, Ryan, Michael T., Ugalde, Cristina [2022]. Two independent respiratory chains adapt OXPHOS performance to glycolytic switch. Cell Metab. 2022 Nov 1;34(11):1792-1808.e6.

Luque, Juan, Mendes, Ingrid, Gómez, Beatriz, Morte, Beatriz, López de Heredia, Miguel, Herreras, Enrique, Corrochano, Virginia, Bueren, Juan, Gallano, Pía, Artuch, Rafael, Fillat, Cristina, Pérez-Jurado, Luis A., Montoliu, Lluis, Carracedo, Ángel, Millán, José M., Webb, Susan M., Palau, Francesc, The CIBERER Network, Lapunzina, Pablo [2022]. CIBERER: Spanish national network for research on rare diseases: A highly productive collaborative initiative. Clin Genet. 2022 May;101(5-6):481-493.

Illescas, María , Peñas, Ana , Arenas, Joaquín , Martín, Miguel A. , Ugalde, Cristina [2021]. Regulation of Mitochondrial Function by the Actin Cytoskeleton. Front Cell Dev Biol. 2021 Dec 21;9:795838.

van Strien, Joeri, Haupt, Alexander, Schulte, Uwe, Braun, Hans-Peter, Cabrera-Orefice, Alfredo, Choudhary, Jyoti S., Evers, Felix, Fernandez-Vizarra, Erika, Guerrero-Castillo, Sergio, Kooij, Taco W.A., Páleníková, Petra, Pardo, Mercedes, Ugalde, Cristina, Wittig, Ilka, Wöhlbrand, Lars, Brandt, Ulrich, Arnold, Susanne, Huynen, Martijn A. [2021]. CEDAR, an online resource for the reporting and exploration of complexome profiling data. Biochim Biophys Acta Bioenerg. 2021 Jul 1;1862(7):148411.

Fernández-Vizarra, Erika, López-Calcerrada, Sandra, Formosa, Luke E., Pérez-Pérez, Rafael, Ding, Shujing, Fearnley, Ian M., Arenas, Joaquín, Martín, Miguel A., Zeviani, Massimo, Ryan, Michael T., Ugalde, Cristina [2021]. SILAC-based complexome profiling dissects the structural organization of the human respiratory supercomplexes in SCAFIKO cells. Biochim Biophys Acta Bioenerg. 2021 Jul 1;1862(7):148414.

Peñas, Ana , Fernández-De la Torre, Miguel , Laine-Menéndez, Sara , Lora, David , Illescas, María , García-Bartolomé, Alberto , Morales-Conejo, Montserrat , Arenas, Joaquín , Martín, Miguel A. , Morán, María , Domínguez-González, Cristina , Ugalde, Cristina [2021]. Plasma Gelsolin Reinforces the Diagnostic Value of FGF-21 and GDF-15 for Mitochondrial Disorders. Int J Mol Sci. 2021 Jun 15;22(12):6396.

González-Quintana, Adrián, Trujillo-Tiebas, María J., Fernández-Perrone, Ana L., Blázquez, Alberto, Lucia, Alejandro, Morán, María, Ugalde, Cristina, Arenas, Joaquín, Ayuso, Carmen, Martín, Miguel A. [2020]. Uniparental isodisomy as a cause of mitochondrial complex I respiratory chain disorder due to a novel splicing NDUFS4 mutation. Mol Genet Metab. 2020 Nov;131(3):341-348.

Timón-Gómez, Alba, Pérez-Pérez, Rafael, Nyvltova, Eva, Ugalde, Cristina, Fontanesi, Flavia, Barrientos, Antoni [2020]. Protocol for the Analysis of Yeast and Human Mitochondrial Respiratory Chain Complexes and Supercomplexes by Blue Native Electrophoresis. STAR Protoc. 2020 Sep 18;1(2):100089.

García-Bartolomé, Alberto , Peñas, Ana , Illescas, María , Bermejo, Verónica , López-Calcerrada, Sandra , Pérez-Pérez, Rafael , Marín-Buera, Lorena , Domínguez-González, Cristina , Arenas, Joaquín , Martín, Miguel A. , Ugalde, Cristina [2020]. Altered Expression Ratio of Actin-Binding Gelsolin Isoforms Is a Novel Hallmark of Mitochondrial OXPHOS Dysfunction. Cells. 2020 Aug 19;9(9):1922.

González-Quintana, Adrián , García-Consuegra, Inés , Belanger-Quintana, Amaya , Serrano-Lorenzo, Pablo , Lucia, Alejandro , Blázquez, Alberto , Docampo, Jorge , Ugalde, Cristina , Morán, María , Arenas, Joaquín , Martín, Miguel A. [2020]. Novel NDUFA13 Mutations Associated with OXPHOS Deficiency and Leigh Syndrome: A Second Family Report. Genes (Basel). 2020 Jul 26;11(8):855.

Lobo‐Jarne, Teresa, Pérez‐Pérez, Rafael, Fontanesi, Flavia, Timón‐Gómez, Alba, Wittig, Ilka, Peñas, Ana, Serrano‐Lorenzo, Pablo, García‐Consuegra, Inés, Arenas, Joaquín, Martín, Miguel A, Barrientos, Antoni, Ugalde, Cristina [2020]. Multiple pathways coordinate assembly of human mitochondrial complex IV and stabilization of respiratory supercomplexes. EMBO J. 2020 Jul 15;39(14):e103912.

Timón-Gómez, Alba, Garlich, Joshua, Stuart, Rosemary A., Ugalde, Cristina, Barrientos, Antoni [2020]. Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis. Cell Rep. 2020 May 5;31(5):107607.

Protasoni, Margherita, Pérez‐Pérez, Rafael, Lobo‐Jarne, Teresa, Harbour, Michael E, Ding, Shujing, Peñas, Ana, Diaz, Francisca, Moraes, Carlos T, Fearnley, Ian M, Zeviani, Massimo, Ugalde, Cristina, Fernández‐Vizarra, Erika [2020]. Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV. EMBO J. 2020 Feb 3;39(3):e102817.

Peralta, Susana, González-Quintana, Adrián, Ybarra, Marta, Delmiro, Aitor, Pérez-Pérez, Rafael, Docampo, Jorge, Arenas, Joaquín, Blázquez, Alberto, Ugalde, Cristina, Martín, Miguel A. [2019]. Novel ATAD3A recessive mutation associated to fatal cerebellar hypoplasia with multiorgan involvement and mitochondrial structural abnormalities. Mol Genet Metab. 2019 Dec;128(4):452-462.