title: Unraveling the complexity of mitochondrial complex I assembly: A dynamic process.
abstract: Mammalian complex I is composed of 44 different subunits and its assembly requires at least 13 specific assembly factors. Proper function of the mitochondrial respiratory chain enzyme is of crucial importance for cell survival due to its major participation in energy production and cell signaling. Complex I assembly depends on the coordination of several crucial processes that need to be tightly interconnected and orchestrated by a number of assembly factors. The understanding of complex I assembly evolved from simple sequential concept to the more sophisticated modular assembly model describing a convoluted process. According to this model, the different modules assemble independently and associate afterwards with each other to form the final enzyme. In this review, we aim to unravel the complexity of complex I assembly and provide the latest insights in this fundamental and fascinating process. This article is part of a Special Issue entitled Respiratory complex I, edited by Volker Zickermann and Ulrich Brandt.
authors.affiliation: Radboud Center for Mitochondrial Medicine, Department of Pediatrics, Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The Netherlands.
authors.forename: Laura
authors.initials: L
authors.lastname: Sánchez-Caballero
authors.affiliation: Radboud Center for Mitochondrial Medicine, Department of Pediatrics, Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The Netherlands.
authors.forename: Sergio
authors.initials: S
authors.lastname: Guerrero-Castillo
authors.affiliation: Radboud Center for Mitochondrial Medicine, Department of Pediatrics, Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The Netherlands.
authors.forename: Leo
authors.initials: L
authors.lastname: Nijtmans
mesh_terms: D000818:Animals; D001665:Binding Sites; D042967:Electron Transport Complex I / Q000737:chemistry* / Q000648:ultrastructure*; D004789:Enzyme Activation; D006801:Humans; D024101:Mitochondrial Proteins / Q000737:chemistry* / Q000648:ultrastructure*; D008956:Models, Chemical; D056004:Molecular Dynamics Simulation; D011485:Protein Binding; D011487:Protein Conformation
chemical_list: D024101:Mitochondrial Proteins; D042967:Electron Transport Complex I
keywords: Assembly factors; Complex I assembly; Human complex I; Mitochondria; Mitochondrial disorders