Reduced cellular cholesterol content in peroxisome-deficient fibroblasts is associated with impaired uptake of the patient's low density lipoprotein and with reduced cholesterol synthesis.


Mammalian cells acquire cellular cholesterol by de novo synthesis as well as by uptake of low density lipoprotein (LDL). Peroxisomes contain enzymes involved in the synthesis of cholesterol, and peroxisome-deficient (PD) patients have been shown to have hypocholesterolemia and abnormal LDL. We therefore decided to study whether cholesterol synthesis and… (More)


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