title: Dual role of mitofilin in mitochondrial membrane organization and protein biogenesis.
abstract: The mitochondrial inner membrane consists of two domains, inner boundary membrane and cristae membrane that are connected by crista junctions. Mitofilin/Fcj1 was reported to be involved in formation of crista junctions, however, different views exist on its function and possible partner proteins. We report that mitofilin plays a dual role. Mitofilin is part of a large inner membrane complex, and we identify five partner proteins as constituents of the mitochondrial inner membrane organizing system (MINOS) that is required for keeping cristae membranes connected to the inner boundary membrane. Additionally, mitofilin is coupled to the outer membrane and promotes protein import via the mitochondrial intermembrane space assembly pathway. Our findings indicate that mitofilin is a central component of MINOS and functions as a multifunctional regulator of mitochondrial architecture and protein biogenesis.
authors.affiliation: Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, 79104 Freiburg, Germany.
authors.forename: Karina
authors.initials: K
authors.lastname: von der Malsburg
authors.forename: Judith M
authors.initials: JM
authors.lastname: Müller
authors.forename: Maria
authors.initials: M
authors.lastname: Bohnert
authors.forename: Silke
authors.initials: S
authors.lastname: Oeljeklaus
authors.forename: Paulina
authors.initials: P
authors.lastname: Kwiatkowska
authors.forename: Thomas
authors.initials: T
authors.lastname: Becker
authors.forename: Adrianna
authors.initials: A
authors.lastname: Loniewska-Lwowska
authors.forename: Sebastian
authors.initials: S
authors.lastname: Wiese
authors.forename: Sanjana
authors.initials: S
authors.lastname: Rao
authors.forename: Dusanka
authors.initials: D
authors.lastname: Milenkovic
authors.forename: Dana P
authors.initials: DP
authors.lastname: Hutu
authors.forename: Ralf M
authors.initials: RM
authors.lastname: Zerbes
authors.forename: Agnes
authors.initials: A
authors.lastname: Schulze-Specking
authors.forename: Helmut E
authors.initials: HE
authors.lastname: Meyer
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Martinou
authors.forename: Sabine
authors.initials: S
authors.lastname: Rospert
authors.forename: Peter
authors.initials: P
authors.lastname: Rehling
authors.forename: Chris
authors.initials: C
authors.lastname: Meisinger
authors.forename: Marten
authors.initials: M
authors.lastname: Veenhuis
authors.forename: Bettina
authors.initials: B
authors.lastname: Warscheid
authors.forename: Ida J
authors.initials: IJ
authors.lastname: van der Klei
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Pfanner
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Chacinska
authors.forename: Martin
authors.initials: M
authors.lastname: van der Laan
mesh_terms: D002846:Chromatography, Affinity; D006801:Humans; D007425:Intracellular Membranes / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism*; D033681:Mitochondrial Membrane Transport Proteins / Q000378:metabolism*; D051336:Mitochondrial Membranes / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism*; D011485:Protein Binding; D014176:Protein Biosynthesis; D021381:Protein Transport; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000254:growth & development; D029701:Saccharomyces cerevisiae Proteins / Q000378:metabolism*; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; D019895:Transposases / Q000378:metabolism
chemical_list: D033681:Mitochondrial Membrane Transport Proteins; D024101:Mitochondrial Proteins; D029701:Saccharomyces cerevisiae Proteins; C473499:Minos transposase; D019895:Transposases