title: Mitofilin complexes: conserved organizers of mitochondrial membrane architecture.
abstract: Mitofilin proteins are crucial organizers of mitochondrial architecture. They are located in the inner mitochondrial membrane and interact with several protein complexes of the outer membrane, thereby generating contact sites between the two membrane systems of mitochondria. Within the inner membrane, mitofilins are part of hetero-oligomeric protein complexes that have been termed the mitochondrial inner membrane organizing system (MINOS). MINOS integrity is required for the maintenance of the characteristic morphology of the inner mitochondrial membrane, with an inner boundary region closely apposed to the outer membrane and cristae membranes, which form large tubular invaginations that protrude into the mitochondrial matrix and harbor the enzyme complexes of the oxidative phosphorylation machinery. MINOS deficiency comes along with a loss of crista junction structures and the detachment of cristae from the inner boundary membrane. MINOS has been conserved in evolution from unicellular eukaryotes to humans, where alterations of MINOS subunits are associated with multiple pathological conditions.
authors.affiliation: Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, D-79104 Freiburg, Germany.
authors.forename: Ralf M
authors.initials: RM
authors.lastname: Zerbes
authors.forename: Ida J
authors.initials: IJ
authors.lastname: van der Klei
authors.forename: Marten
authors.initials: M
authors.lastname: Veenhuis
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Pfanner
authors.forename: Martin
authors.initials: M
authors.lastname: van der Laan
authors.forename: Maria
authors.initials: M
authors.lastname: Bohnert
mesh_terms: D000818:Animals; D017124:Conserved Sequence; D006801:Humans; D051336:Mitochondrial Membranes / Q000737:chemistry / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000737:chemistry* / Q000378:metabolism*; D008954:Models, Biological; D009124:Muscle Proteins / Q000737:chemistry / Q000378:metabolism
chemical_list: C088548:IMMT protein, human; D024101:Mitochondrial Proteins; D009124:Muscle Proteins