Mitochondrial contact site and cristae organizing system: A central player in membrane shaping and crosstalk

• Published in: Biochimica et biophysica acta. Molecular cell research Vol. 1864; no. 9; pp. 1481 - 1489
• Main Authors: Wollweber, Florian, von der Malsburg, Karina, van der Laan, Martin
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.09.2017
• Subjects: Animals; Contact sites; Cristae; Humans; Membrane organization; MICOS; Mitochondria; Mitochondria - metabolism; Mitochondria - ultrastructure; Mitochondrial Membrane Transport Proteins - genetics; Mitochondrial Membrane Transport Proteins - metabolism; Mitochondrial Membranes - metabolism; Signal Transduction; Mitochondria; TOM; MIA; MICOS; OPA1; MicX; Membrane organization; Cristae; TIM23; SAM; Contact sites
• ISSN: 0167-4889; 1879-2596
• DOI: 10.1016/j.bbamcr.2017.05.004

Mitochondria are multifunctional metabolic factories and integrative signaling organelles of eukaryotic cells. The structural basis for their numerous functions is a complex and dynamic double-membrane architecture. The outer membrane connects mitochondria to the cytosol and other organelles. The inner membrane is composed of a boundary region and highly folded cristae membranes. The evolutionarily conserved mitochondrial contact site and cristae organizing system (MICOS) connects the two inner membrane domains via formation and stabilization of crista junction structures. Moreover, MICOS establishes contact sites between inner and outer mitochondrial membranes by interacting with outer membrane protein complexes. MICOS deficiency leads to a grossly altered inner membrane architecture resulting in far-reaching functional perturbations in mitochondria. Consequently, mutations affecting the function of MICOS are responsible for a diverse spectrum of human diseases. In this article, we summarize recent insights and concepts on the role of MICOS in the organization of mitochondrial membranes. This article is part of a Special Issue entitled: Membrane Contact Sites edited by Christian Ungermann and Benoit Kornmann. •Mitochondria exhibit a particularly complex double-membrane architecture.•Central role of the mitochondrial contact site and cristae organizing system (MICOS)•MICOS shapes crista junctions and mitochondrial membrane contact sites.•MICOS deficiency causes structural and functional perturbations in mitochondria.