A 5-year-old girl exhibits a progressive decline in motor and sensory functions and is quickly reduced to a vegetative state. MRI reveals confluent, bilaterally symmetric, demyelination. At autopsy,...

A 5-year-old girl exhibits a progressive decline in motor and sensory functions and is quickly reduced to a vegetative state. MRI reveals confluent, bilaterally symmetric, demyelination. At autopsy, the brain shows gliosis and perivascular infiltrates of lymphocytes. Laboratory studies disclose high levels of saturated very long–chain fatty acids in tissues and body fluids. This patient’s leukodystrophy is most likely caused by an inborn error in the function of what cell component?

(A) Golgi apparatus


(B) Mitochondria


(C) Nuclear envelope


(D) Peroxisome


(E) Plasma membrane



May 24, 2022
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