Metabolic function and architecture of mitochondria are intimately linked. More than 60 years ago, cristae were discovered as characteristic elements of mitochondria that harbor the protein complexes of oxidative phosphorylation, but how cristae are formed, remained an open question. Here we present experimental results obtained with yeast that support a novel hypothesis on the existence of two molecular pathways that lead to the generation of lamellar and tubular cristae. Formation of lamellar cristae depends on the mitochondrial fusion machinery through a pathway that is required also for homeostasis of mitochondria and mitochondrial DNA. Tubular cristae are formed via invaginations of the inner boundary membrane by a pathway independent of the fusion machinery. Dimerization of the F1FO-ATP synthase and the presence of the MICOS complex are necessary for both pathways. The proposed hypothesis is suggested to apply also to higher eukaryotes, since the key components are conserved in structure and function throughout evolution.DOI: http://dx.doi.org/10.7554/eLife.18853.001
Structure and function of mitochondria are intimately linked. In a search for components that participate in building the elaborate architecture of this complex organelle we have identified Aim24, an inner membrane protein. Aim24 interacts with the MICOS complex that is required for the formation of crista junctions and contact sites between inner and outer membranes. Aim24 is necessary for the integrity of the MICOS complex, for normal respiratory growth and mitochondrial ultrastructure. Modification of MICOS subunits Mic12 or Mic26 by His-tags in the absence of Aim24 leads to complete loss of cristae and respiratory complexes. In addition, the level of tafazzin, a cardiolipin transacylase, is drastically reduced and the composition of cardiolipin is modified like in mutants lacking tafazzin. In conclusion, Aim24 by interacting with the MICOS complex plays a key role in mitochondrial architecture, composition and function.DOI: http://dx.doi.org/10.7554/eLife.01684.001
The budding yeast Saccharomyces cerevisiae is an important model organism to study cellular structure and function. Due to its excellent accessibility to genetics and biochemical and microscopic analyses, studies with yeast have provided fundamental insights into mitochondrial biology. Yeast offers additional advantages because it can grow under fermenting conditions when oxidative phosphorylation is not obligatory and because the majority of mitochondrial structure and function are largely conserved during evolution. Isolation of mitochondria is an important technique for mitochondrial studies. This chapter focuses on procedures for the isolation and purification of intact yeast mitochondria that can be used for numerous functional assays as well as for analyses of mitochondrial ultrastructure.
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