A newborn infant has severe respiratory problems. Over the next few days, it is observed that the baby has severe muscle problems, demonstrates little development, and has neurological problems. A liver biopsy reveals a very low level of acetyl CoA carboxylase, but normal levels of the enzymes of glycolysis, gluconeogenesis, the citric acid cycle, and the pentose phosphate pathway. What is the most likely cause of the infant’s respiratory problems?
Correct Answer: Low levels of phosphatidyl choline
Description: Acetyl CoA carboxylase deficiency drastically alters the ability of the patient to synthesize fatty acids. The fact that the infant was born at all is due to the body's ability to utilize fatty acids provided to it. However, all processes dependent upon de novo fatty acid biosynthesis are affected. The lungs, in paicular, require surfactant, a lipoprotein substance secreted by alveolar type II cells, to function properly. Surfactant lowers alveolar surface tension, facilitating gas exchange. It contains significant amounts of dipalmitoyl phosphatidylcholine. Palmitate is the major end product of de novo fatty acid synthesis. Acetyl CoA carboxylase formation of malonyl CoA is the first step of fatty acid synthesis. Biotin deficiency cannot be the problem because pyruvate carboxylase in gluconeogenesis is not affected. None of the other answers listed would result in all of the symptoms given.
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