The mitochondrial contact site complex, a determinant of mitochondrial architecture
Open Access
- 18 October 2011
- journal article
- research article
- Published by Springer Science and Business Media LLC in The EMBO Journal
- Vol. 30 (21), 4356-4370
- https://doi.org/10.1038/emboj.2011.379
Abstract
Mitochondria are organelles with a complex architecture. They are bounded by an envelope consisting of the outer membrane and the inner boundary membrane (IBM). Narrow crista junctions (CJs) link the IBM to the cristae. OMs and IBMs are firmly connected by contact sites (CS). The molecular nature of the CS remained unknown. Using quantitative high‐resolution mass spectrometry we identified a novel complex, the mitochondrial contact site (MICOS) complex, formed by a set of mitochondrial membrane proteins that is essential for the formation of CS. MICOS is preferentially located at the CJs. Upon loss of one of the MICOS subunits, CJs disappear completely or are impaired, showing that CJs require the presence of CS to form a superstructure that links the IBM to the cristae. Loss of MICOS subunits results in loss of respiratory competence and altered inheritance of mitochondrial DNA.Keywords
This publication has 61 references indexed in Scilit:
- Deep and Highly Sensitive Proteome Coverage by LC-MS/MS Without PrefractionationMolecular & Cellular Proteomics, 2011
- Lateral release of proteins from the TOM complex into the outer membrane of mitochondriaThe EMBO Journal, 2011
- ChChd3, an Inner Mitochondrial Membrane Protein, Is Essential for Maintaining Crista Integrity and Mitochondrial FunctionOnline Journal of Public Health Informatics, 2011
- Formation of cristae and crista junctions in mitochondria depends on antagonism between Fcj1 and Su e/gThe Journal of cell biology, 2009
- MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantificationNature Biotechnology, 2008
- Dimer ribbons of ATP synthase shape the inner mitochondrial membraneThe EMBO Journal, 2008
- Dynamic subcompartmentalization of the mitochondrial inner membraneThe Journal of cell biology, 2006
- Blue native PAGENature Protocols, 2006
- Proteomic characterization of the human centrosome by protein correlation profilingNature, 2003
- Zones of membrane adhesion in the cryofixed envelope of Escherichia coliJournal of Structural Biology, 1991