By Carolyn D Berdanier
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Extra info for Mitochondria in health and disease
Example text
The wrinkles of the inner membrane form lamellar structures called cristae. On the inner membrane (facing into the matrix) are numerous small spheres on stalks, which are the structures associated with the multisubunit ATP synthetase or the F1F0ATPase. The inner membrane is permeable only to water, oxygen, and carbon dioxide and is impermeable to a wide variety of molecules and ions that, in turn, require specific transporters for entry and exit. A number of transport systems and enzymes are in the inner membrane, thus accounting for the fact that this membrane consists of ~80% by weight protein and only 20% lipid.
Once the fatty acid is activated, it is bound to carnitine with the release of CoA. The acyl carnitine is then translocated through both mitochondrial membranes into the mitochondrial matrix via the carnitine acylcarnitine translocase. As one molecule of acylcarnitine is passed into the matrix, one molecule of carnitine is translocated back to the cytosol and the acylcarnitine is converted back to acyl CoA. 5. 4 Initial steps in the oxidation of fatty acids by the mitochondrial enzymes. Without carnitine, the oxidation of fatty acids, especially the long chain fatty acids, cannot proceed.
The enzymes of the respiratory chain are arranged so Introduction to Mitochondria 41 as to transport hydrogen ions from the matrix across the inner membrane. When this occurs, a proton gradient develops in close proximity to the F1F0ATPase (ATP synthase) complex and provides sufficient energy to drive ATP synthesis by causing a dehydration of ADP and Pi. Reducing equivalents transported into the mitochondrial compartment by the various substrate shuttle systems or generated in the compartment are passed down the respiratory chain in carefully regulated steps.