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Impaired myelin formation in experimental hyperphenylalaninaemia
Authors:S N Shah  N A Peterson  C M McKean
Abstract:Abstract— From the 5th to the 20th postnatal day, rats were injected with saline (controls) or phenylalanine (3 mg/g daily). The quantities of cholesterol and glycolipids were significantly lower in the phenylalanine-injected rats, but brain DNA and protein contents were the same in both groups. As a result of the reduced formation of lipids, the hyperphenylalaninaemic brains contained significantly less myelin than control brains, but the gross composition [ratio of protein: cholesterol: galactolipid: phospholipid) of the myelin was unchanged. We suggest that in hyperphenylalaninaemic brains the reduced availability of cholesterol may cause a reduction in the formation of cholesterol-phospholipid-protein complexes in brain, which in turn may limit the formation of myelin.
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