BIOSS
Centre for Biological Signalling Studies

Uniform nomenclature for the mitochondrial contact site and cristae organizing system

31.03.2014

Pfanner N, van der Laan M, Amati P, Capaldi RA, Caudy AA, Chacinska A, Darshi M, Deckers M, Hoppins S, Icho T, Jakobs S, Ji J, Kozjak-Pavlovic V, Meisinger C, Odgren PR, Park SK, Rehling P, Reichert AS, Sheikh MS, Taylor SS, Tsuchida N, van der Bliek AM, van der Klei IJ, Weissman JS, Westermann B, Zha J, Neupert W, Nunnari J.

J Cell Biol. 2014;204(7):1083-6

J Cell Biol         online article

The inner membrane of mitochondria is composed of two distinct subdomains, the inner boundary membrane and the highly folded cristae, with defined protein compositions and functions. Both inner membrane subdomains are connected by crista junction structures. A central component required for the formation and maintenance of crista junctions is the evolutionary conserved mitochondrial contact site and cristae organizing system (MICOS complex). Mutations in different organisms that inactivate the multi-subunit MICOS complex lead to a detachment of cristae membranes from the inner boundary membrane. Moreover, MICOS is involved in the formation of contact sites between inner and outer mitochondrial membranes. Thus, MICOS represents a key player in the generation of the typical mitochondrial architecture that supports a plethora of physiologically important functions.