Mitofilin complexes: conserved organizers of mitochondrial membrane architecture
01.11.2012
Zerbes RM, van der Klei IJ, Veenhuis M, Pfanner N, van der Laan M, Bohnert M.
Biol Chem. 2012;393(11):1247-61
Mitochondria are surrounded by two membranes, an outer envelope membrane and a highly folded inner membrane. The characteristic invaginations of the inner mitochondrial membrane, termed cristae, are connected to the boundary inner membrane by morphologically well defined structures, the crista junctions. The recently discovered Mitochondrial Inner Membrane Organizing System (MINOS) is required for the maintenance of crista junctions. Mutations that inactivate MINOS lead to a detachment of cristae membranes that accumulate as extended, stacked membrane sheets in the mitochondrial matrix. In this article we discuss the structure and function of MINOS complexes in different organisms and summarize the multiple links between MINOS defects and human diseases.
