You are called in as a consult for a patient who suffers from acidosis. Urine levels of both lactic acid and isocitric acid are elevated. Mitochondria isolated from the patient’s fibroblasts oxidize pyruvate very slowly whereas α-ketoglutarate and malate are oxidized normally. a. At what enzyme would you predict the patient has a defect? b. Why? What evidence do you have to choose that enzyme? c. How do you account for the increase in lactic acid?

Organic And Biological Chemistry
7th Edition
ISBN:9781305081079
Author:STOKER, H. Stephen (howard Stephen)
Publisher:STOKER, H. Stephen (howard Stephen)
Chapter10: Enzymes And Vitamins
Section: Chapter Questions
Problem 10.43EP
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You are called in as a consult for a patient who suffers from acidosis. Urine levels of both lactic acid and isocitric acid are elevated. Mitochondria isolated from the patient’s fibroblasts oxidize pyruvate very slowly whereas α-ketoglutarate and malate are oxidized normally. a. At what enzyme would you predict the patient has a defect? b. Why? What evidence do you have to choose that enzyme? c. How do you account for the increase in lactic acid?
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