The Mitochondrial Contact Site Complex, a Determinant of Mitochondrial Architecture

Overview

Journal EMBO J

Specialties Biotechnology
Molecular Biology

Date 2011 Oct 20

PMID 22009199

Citations 221

Authors

Max Harner

Christian Korner

Dirk Walther

Dejana Mokranjac

Johannes Kaesmacher

Ulrich Welsch

Janice Griffith

Matthias Mann

Fulvio Reggiori

Walter Neupert

Affiliations

Soon will be listed here.

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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