title: The MIA system for protein import into the mitochondrial intermembrane space.
abstract: When thinking of the mitochondrial intermembrane space we envisage a small compartment that is bordered by the mitochondrial outer and inner membranes. Despite this somewhat simplified perception the intermembrane space has remained a central focus in mitochondrial biology. This compartment accommodates many proteinaceous factors that play critical roles in mitochondrial and cellular metabolism, including the regulation of programmed cell death and energy conversion. The mechanism by which intermembrane space proteins are transported into the organelle and folded remained largely unknown until recently. In pursuit of the answer to this question a novel machinery, the Mitochondrial Intermembrane Space Assembly machinery, exploiting a unique regulated thiol-disulfide exchange mechanism has been revealed. This exciting discovery has not only put in place novel concepts for the biogenesis of intermembrane space precursors but also raises important implications on the mechanisms involved in the generation and transfer of disulfide bonds.
authors.affiliation: Institut für Biochemie und Molekularbiologie, Zentrum für Biochemie und Molekulare Zellforschung, Universität Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany.
authors.forename: Diana
authors.initials: D
authors.lastname: Stojanovski
authors.forename: Judith M
authors.initials: JM
authors.lastname: Müller
authors.forename: Dusanka
authors.initials: D
authors.lastname: Milenkovic
authors.forename: Bernard
authors.initials: B
authors.lastname: Guiard
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Pfanner
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Chacinska
mesh_terms: D007425:Intracellular Membranes / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism*; D033681:Mitochondrial Membrane Transport Proteins / Q000378:metabolism*; D051336:Mitochondrial Membranes / Q000378:metabolism*; D021381:Protein Transport
chemical_list: D033681:Mitochondrial Membrane Transport Proteins