HMGCR and phenylketonuria: Although in this study we have only analyzed the blood lipid profile of patients with PKU, it is worth mentioning that an in vivo study, in which experimental hyperphenylalaninemia was induced in an animal model, demonstrated that there was an inhibition of two of the main regulatory enzymes of brain and liver cholesterogenesis: 3-hydroxy-3-methylglutaryl-CoA reductase and mevalonate-5-pyrophosphate decarboxylase [29]; and therefore a reduced cholesterol synthesis in the brain may indicate an association between impaired myelination and mental retardation in patients with PKU.