Abstract

Publisher Summary This chapter describes the mitochondrion as an intracellular organelle found in virtually all eukaryotic cells, where it plays a major role in cellular ATP production. It consists of four compartments; the inner and outer membranes and two soluble fractions, the matrix and the intermembrane space. The inner membrane and matrix are associated with most of the functional activities of the mitochondria, including those involved with the tricarboxylic acid (TCA) cycle, fatty acid oxidation and ATP generation. The inner membrane is folded forming cristae giving it a much larger surface area than the outer membrane. The main function of the mitochondrion is ATP synthesis. The enzymes for the TCA cycle and β-oxidation of fatty acids are situated in the mitochondrial matrix where dicarboxylic, tricarboxylic, and fatty acids are oxidized generating NADH and FADH2. The chapter discusses that the majority of mitochondrial proteins are encoded by the nucleus, synthesized on cytoribosomes and imported into the mitochondrion. However, mitochondria contain their own DNA (mtDNA), which is circular and double stranded.

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