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Publicly Available Published by De Gruyter October 11, 2012

Mitofilin complexes: conserved organizers of mitochondrial membrane architecture

Ralf M. Zerbes

Ralf M. Zerbes studied Molecular Medicine at the Universities of Freiburg and Perth. He is now a PhD student in the group of Martin van der Laan working on the molecular architecture and function of the MINOS complex

, Ida J. van der Klei

Ida J. van der Klei, PhD, was trained at the Universities of Groningen (RUG) and Munich. Since 2005 chair of Molecular Cell Biology and since 2007 full Professor at the RUG. Research areas: molecular cell biology of yeast and filamentous fungi, cell organelles, peroxisome biogenesis and quality control

, Marten Veenhuis

Marten Veenhuis, PhD, obtained his education at the University of Groningen (RUG). Founder and chair of the research group Molecular Cell Biology (formerly Eukaryotic Microbiology) at the RUG, now emeritus. Research areas: peroxisome biology, yeast, filamentous fungi, fluorescence and electron microscopy

, Nikolaus Pfanner

Nikolaus Pfanner, MD, was trained at the Universities of Munich and Princeton. Since 1992 Professor and Chair of Biochemistry at the University of Freiburg. Research areas: Protein sorting in cells, biogenesis of mitochondria, membrane protein networks

, Martin van der Laan

Martin van der Laan, PhD, received his professional training at the Universities of Osnabrück and Groningen. Since 2007 group leader (Assistant Professor) at the University of Freiburg. Research areas: Intracellular protein sorting, biogenesis and dynamics of organellar membranes

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and Maria Bohnert

Maria Bohnert, PhD, studied Molecular Medicine at the University of Freiburg and performed her PhD work on mitochondrial protein biogenesis supported by a Boehringer Ingelheim Fonds fellowship. She is currently working as a post-doc in the group of Martin van der Laan, where she studies the principles of mitochondrial membrane organization

From the journal Biological Chemistry

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.


Corresponding author: Martin van der Laan, Institut für Biochemie und Molekularbiologie, ZBMZ, Universität Freiburg, D-79104 Freiburg, Germany; and BIOSS Centre for Biological Signalling Studies, Universität Freiburg, D-79104 Freiburg, Germany

About the authors

Ralf M. Zerbes

Ralf M. Zerbes studied Molecular Medicine at the Universities of Freiburg and Perth. He is now a PhD student in the group of Martin van der Laan working on the molecular architecture and function of the MINOS complex

Ida J. van der Klei

Ida J. van der Klei, PhD, was trained at the Universities of Groningen (RUG) and Munich. Since 2005 chair of Molecular Cell Biology and since 2007 full Professor at the RUG. Research areas: molecular cell biology of yeast and filamentous fungi, cell organelles, peroxisome biogenesis and quality control

Marten Veenhuis

Marten Veenhuis, PhD, obtained his education at the University of Groningen (RUG). Founder and chair of the research group Molecular Cell Biology (formerly Eukaryotic Microbiology) at the RUG, now emeritus. Research areas: peroxisome biology, yeast, filamentous fungi, fluorescence and electron microscopy

Nikolaus Pfanner

Nikolaus Pfanner, MD, was trained at the Universities of Munich and Princeton. Since 1992 Professor and Chair of Biochemistry at the University of Freiburg. Research areas: Protein sorting in cells, biogenesis of mitochondria, membrane protein networks

Martin van der Laan

Martin van der Laan, PhD, received his professional training at the Universities of Osnabrück and Groningen. Since 2007 group leader (Assistant Professor) at the University of Freiburg. Research areas: Intracellular protein sorting, biogenesis and dynamics of organellar membranes

Maria Bohnert

Maria Bohnert, PhD, studied Molecular Medicine at the University of Freiburg and performed her PhD work on mitochondrial protein biogenesis supported by a Boehringer Ingelheim Fonds fellowship. She is currently working as a post-doc in the group of Martin van der Laan, where she studies the principles of mitochondrial membrane organization

Received: 2012-6-25
Accepted: 2012-7-9
Published Online: 2012-10-11
Published in Print: 2012-11-01

©2012 by Walter de Gruyter Berlin Boston

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