Journal
EMBO JOURNAL
Volume 30, Issue 21, Pages 4356-4370Publisher
WILEY
DOI: 10.1038/emboj.2011.379
Keywords
contact site proteins; crista junction; MICOS; mitochondrial membrane proteome; molecular architecture of mitochondria
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Funding
- Deutsche Forschungsgemeinschaft [SFB 594]
- Netherlands Organization for Health Research and Development [ZonMW-VIDI-917.76.329]
- Netherlands Organization for Scientific Research [700.59.003]
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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. The EMBO Journal (2011) 30, 4356-4370. doi:10.1038/emboj.2011.379; Published online 18 October 2011
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