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The mitochondrial contact site complex, a determinant of mitochondrial architecture
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The mitochondrial contact site complex, a determinant of mitochondrial architecture
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Journal Article
The mitochondrial contact site complex, a determinant of mitochondrial architecture
2011
Overview
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
mi
tochondrial
co
ntact
s
ite (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 outer and inner mitochondrial membranes are physically linked. Quantitative high resolution mass spectrometry now identifies the molecular nature of the Mitochondrial Contact Site complex (MICOS). MICOS is required for crista junctions formation, respiration and mitochondrial DNA inheritance.
Publisher
John Wiley & Sons, Ltd,Nature Publishing Group UK,Springer Nature B.V,Nature Publishing Group
Subject
/ DNA, Mitochondrial - metabolism
/ EMBO05
/ EMBO20
/ EMBO21
/ Intracellular Membranes - metabolism
/ Membrane Proteins - genetics
/ Membrane Proteins - metabolism
/ MICOS
/ Mitochondria - ultrastructure
/ mitochondrial membrane proteome
/ Mitochondrial Proteins - chemistry
/ Mitochondrial Proteins - metabolism
/ Mitochondrial Proteins - physiology
/ molecular architecture of mitochondria
/ Multiprotein Complexes - chemistry
/ Multiprotein Complexes - metabolism
/ Multiprotein Complexes - physiology
/ Organisms, Genetically Modified
/ Protein Binding - physiology
/ Saccharomyces cerevisiae - genetics
