Figure 1

The form and functions of mitochondria. (a) In yeast, mitochondria form a connected, tubular network that is evenly distributed at the cell cortex. (b) Mitochondria also form well-distributed tubular networks in a majority of mammalian cell types. The mitochondrial network of a mouse embryonic fibroblast is shown. Scale bar, 2 μ for (a,b). (c) Like their bacterial ancestors, mitochondria possess two structurally and functionally distinct membranes, the mitochondrial outer and inner membranes (MOM and MIM, respectively). The MOM and MIM surround two compartments, the inner membrane space and matrix, respectively. The matrix houses the circular mitochondrial genome (mtDNA), which encodes protein components of the respiratory complexes I to IV. The MIM, the most protein dense membrane in the cell, adopts elaborate folds called cristae in which assembled respiratory complexes are housed. In addition to ATP production via oxidative phosphorylation, mitochondria play critical roles in phospholipid biosynthesis, metabolite exchange/buffering, β-oxidation of fatty acids, iron-sulfur cluster biogenesis, pyrimidine biosynthesis and the storage and release of apoptotic factors (reviewed in [1]). TCA, tricarboxylic acid.