The physicians decided to measure the oxygen consumption using intact pure mitoc

Question

The physicians decided to measure the oxygen consumption using intact pure mitochondria isolated from a biopsy of the patient's muscle tissue. A student volunteered to make the measurements, comparing the patient's mitochondria with the mitochondria isolated from the muscle tissue of a normal control. The student checked each individual citric acid cycle enzyme and found that the rate of oxygen consumption was about 25% of the control value using succinate as substrate, but was normal with fumarate and malate. 3.What appears to be the primary defect in the patient?

Explanation / Answer

The patient is having the deficiency of enzye succinate dehydrogenase. This enzyme occurs in eukaryotes and also in bacteria found in respiratory enzyme complex II. it occurs in inner mitochondrial membrane and high amounts of succinate dehydrogenase in muscle results in high mitochondrial content and exhibits high oxidative potential. The enzyme succinate dehydrogenase carries out the oxidation of succinate to fumarate. Deficiency of this enzyme results in only 25% of the rate of consumption of oxygen compared with control. While, the other substrates fumarate and malate exhibited normal rate of consumption. Therefore, the primary defect in patient is the enzyme succinate dehydrogenase.

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