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5. You are asked to determine whether a chemical is an electron-transport-chain inhibitor or an inhibitor of ATP synthase. Design an experiment to determine this. 6. A mutation in a mitochondrial gene encoding a component of ATP synthase has been identified. People who have this mutation suffer from muscle weakness, neurological symptoms, and difficulty with their vision. A tissue biopsy was performed on each patient, and submitochondrial particles were isolated that were capable of succinate-sustained ATP synthesis. The activity of the ATP synthase was measured on the addition of succinate and the following results were obtained. ATP synthase activity (nmol of ATP formed min^-1 mg^-1) Controls 3.0 Patient 1 0.25 Patient 2 0.11 Patient 3 0.17 a. Why was succinate added? b. What is the effect of the mutation on ATP synthase activity?

          5. You are asked to determine whether a chemical is an electron-transport-chain inhibitor or an inhibitor of ATP synthase. Design an experiment to determine this. 6. A mutation in a mitochondrial gene encoding a component of ATP synthase has been identified. People who have this mutation suffer from muscle weakness, neurological symptoms, and difficulty with their vision. A tissue biopsy was performed on each patient, and submitochondrial particles were isolated that were capable of succinate-sustained ATP synthesis. The activity of the ATP synthase was measured on the addition of succinate and the following results were obtained. ATP synthase activity (nmol of ATP formed min^-1 mg^-1) Controls 3.0 Patient 1 0.25 Patient 2 0.11 Patient 3 0.17 a. Why was succinate added? b. What is the effect of the mutation on ATP synthase activity?

5. You are asked to determine whether a chemical is an electron-transport-chain inhibitor or an inhibitor of ATP synthase. Design an experiment to determine this. 6. A mutation in a mitochondrial gene encoding a component of ATP synthase has been identified. People who have this mutation suffer from muscle weakness, neurological symptoms, and difficulty with their vision. A tissue biopsy was performed on each patient, and submitochondrial particles were isolated that were capable of succinate-sustained ATP synthesis. The activity of the ATP synthase was measured on the addition of succinate and the following results were obtained. ATP synthase activity (nmol of ATP formed min^-1 mg^-1) Controls 3.0 Patient 1 0.25 Patient 2 0.11 Patient 3 0.17 a. Why was succinate added? b. What is the effect of the mutation on ATP synthase activity?

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