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

doi:10.1038/emboj.2011.379

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

Research output: Contribution to journal › Article › Academic › peer-review

372 Citations (Scopus)

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.

Original language	English
Pages (from-to)	4356-4370
Number of pages	15
Journal	The EMBO Journal
Volume	30
Issue number	21
DOIs	https://doi.org/10.1038/emboj.2011.379
Publication status	Published - 2-Nov-2011
Externally published	Yes

Keywords

Binding Sites
DNA, Mitochondrial
Intracellular Membranes
Membrane Proteins
Microscopy, Electron
Mitochondria
Mitochondrial Proteins
Models, Biological
Multiprotein Complexes
Organisms, Genetically Modified
Protein Binding
Protein Transport
Saccharomyces cerevisiae

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title = "The mitochondrial contact site complex, a determinant of mitochondrial architecture",

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.",

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AU - Körner, Christian

AU - Walther, Dirk

AU - Mokranjac, Dejana

AU - Kaesmacher, Johannes

AU - Welsch, Ulrich

AU - Griffith, Janice

AU - Mann, Matthias

AU - Reggiori, Fulvio

AU - Neupert, Walter

AU - Reggiori, Fulvio

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AB - 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.

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KW - DNA, Mitochondrial

KW - Intracellular Membranes

KW - Membrane Proteins

KW - Microscopy, Electron

KW - Mitochondria

KW - Mitochondrial Proteins

KW - Models, Biological

KW - Multiprotein Complexes

KW - Organisms, Genetically Modified

KW - Protein Binding

KW - Protein Transport

KW - Saccharomyces cerevisiae

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SP - 4356

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JO - The EMBO Journal

JF - The EMBO Journal

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ER -

The mitochondrial contact site complex, a determinant of mitochondrial architecture

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Keywords

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