Inhibitors of Phosphorylation Effects of Mitochondrial Defects
Amobarbital
Cyanide
Carbon monoxide Myopathy
Encelopathy
Lactic acidosis Enzymes act as markers Mitochondrial Outer membrane: Permeable to most metabolites Presence of enzymes: Acyl CoA synthetase Glycophosphate acyltransferase Intermembrane space Enzymes: Adenylyl kinase Creatine kinase Inner membrane Enclosing a matrix within the mitochondria Phospholipid cardiolipin is concentrated in the inner membrane Enzymes of the respiratory chain ATP synthase Membrane transporters Respiratory Chain Oxides Reducing Equivalents Most of the energy liberated during oxidation of the carbs, fatty acids, and amino acids are made available within the mitochondria as reducing equivalents (-H or electrons) Enzymes of the citric acid cycle and Beta-oxidation are contained within the mitochondria Together with the respiratory chain→ Collects, transports reducing equivalents Direct them to their final reaction with oxygen to form water Drive the process by which liberated energy is trapped as high energy phosphate Components of the Chain: Four Large Protein Complexes in the Inner