Review Question - QID 106611

A 1-week-old infant presents to the ED with seizures that are very difficult to control despite loading with fosphenytoin. The parents note that the child was born at home and has been like this since birth. They note that it has been a difficult week trying to get any response from the infant. Upon examination the infant has poor muscle tone, severe difficulties with sucking and swallowing, corneal opacities, and hepatomegaly. Based on clinical suspicion, a genetic test is performed that reveals the diagnosis. The geneticist counsels that the infant has a rare disorder that causes the accumulation of very long chain fatty acids, adversely affecting myelination and leading to neurological symptoms. Most patients with this disorder die within 1 year. The most likely condition in this patient primarily affects which subcellular compartment?