The mitochondrial contact site complex, a determinant of mitochondrial architecture

Max Harner, Christian Körner, Dirk Walther, Dejana Mokranjac, Johannes Kaesmacher, Ulrich Welsch, Janice Griffith, Matthias Mann, Fulvio Reggiori, Walter Neupert, Fulvio Reggiori

OnderzoeksoutputAcademicpeer review

344 Citaten (Scopus)


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.

Originele taal-2English
Pagina's (van-tot)4356-4370
Aantal pagina's15
TijdschriftThe EMBO Journal
Nummer van het tijdschrift21
StatusPublished - 2-nov.-2011
Extern gepubliceerdJa

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