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Muscle weakness and hypoketotic coma on fasting

Objectives

By the end of this exercise you should be able to:

Explain why the pathway of oxidation of fatty acids to acetyl CoA is called beta-oxidation.

Describe the pathway of beta-oxidation:

dehydrogenation to form a carbon-carbon double bond
addition of water across the carbon-carbon double bond forming a hydroxyl group at the beta-carbon
oxidation of the hydroxyl group to an oxo group
cleavage of the oxo-fatty acyl CoA to yield acetyl CoA and a fatty acyl CoA 2 carbons shorter

Calculate the ATP yield from the oxidation of palmitate (C16:0)

Explain why none of the intermediates of fatty acid oxidation is normally found in tissues

Explain why children with genetic defects of the long-chain fatty acid trifunctional protein develop hypoketotic coma on fasting and outline an appropriate way of treating them.