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Problem- a.1) Muscle biopsy and enzyme assays of a carnitine-deficient individual demonstrate that medium-chain (C8-C10) fatty acids can be metabolized normall, despite the carnitine deficiency. What does this tell you about the role of carnitine in fatty acid transport across the inner mitochondrial membrane?

a.2) Some individuals suffer from medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. Which intermediates would accumulate in individuals with MCAD deficiency?

b) If [3-14C] propionate, 14CH3CH2COO- (i.e., 14C in the methyl group) is added to a liver homogenate, 14C-labeled oxaloacetate is rapidly produced. Draw a flow chart for the pathway, by which propionate is transformed to oxaloacetate. Indicate the location of the 14C in oxaloacetate.

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