title: The protein import machinery of mitochondria-a regulatory hub in metabolism, stress, and disease.
abstract: Mitochondria fulfill central functions in bioenergetics, metabolism, and apoptosis. They import more than 1,000 different proteins from the cytosol. It had been assumed that the protein import machinery is constitutively active and not subject to detailed regulation. However, recent studies indicate that mitochondrial protein import is regulated at multiple levels connected to cellular metabolism, signaling, stress, and pathogenesis of diseases. Here, we discuss the molecular mechanisms of import regulation and their implications for mitochondrial homeostasis. The protein import activity can function as a sensor of mitochondrial fitness and provides a direct means of regulating biogenesis, composition, and turnover of the organelle.
authors.affiliation: Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany; Trinationales Graduiertenkolleg 1478, Universität Freiburg, 79104 Freiburg, Germany; Faculty of Biology, Universität Freiburg, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Universität Freiburg, 79104 Freiburg, Germany.
authors.forename: Angelika B
authors.initials: AB
authors.lastname: Harbauer
authors.affiliation: Leibniz-Institute for Analytical Sciences-ISAS-e.V., 44139 Dortmund, Germany.
authors.forename: René P
authors.initials: RP
authors.lastname: Zahedi
authors.affiliation: Leibniz-Institute for Analytical Sciences-ISAS-e.V., 44139 Dortmund, Germany; Medizinisches Proteom-Center, Ruhr-Universität Bochum, 44801 Bochum, Germany.
authors.forename: Albert
authors.initials: A
authors.lastname: Sickmann
authors.affiliation: Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Universität Freiburg, 79104 Freiburg, Germany. Electronic address: nikolaus.pfanner@biochemie.uni-freiburg.de.
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Pfanner
authors.affiliation: Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Universität Freiburg, 79104 Freiburg, Germany. Electronic address: chris.meisinger@biochemie.uni-freiburg.de.
authors.forename: Chris
authors.initials: C
authors.lastname: Meisinger
mesh_terms: D004734:Energy Metabolism; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism; D011498:Protein Precursors / Q000378:metabolism; D021381:Protein Transport; D015398:Signal Transduction
chemical_list: D024101:Mitochondrial Proteins; D011498:Protein Precursors