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Using a well\u2010defined model for RS, the C57BL6\/<jats:italic>Mecp2<\/jats:italic><jats:sup><jats:italic>tm1.1Bird<\/jats:italic><\/jats:sup> mouse, we have previously found a moderate but persistently lower rate of cholesterol synthesis, measured <jats:italic>in vivo<\/jats:italic>, in the brains of <jats:italic>Mecp2<\/jats:italic><jats:sup><jats:italic>\u2212\/y<\/jats:italic><\/jats:sup> mice, starting from about the third week after birth. There was no genotypic difference in the total cholesterol concentration throughout the brain at any age. This raised the question of whether the lower rate of cholesterol synthesis in the mutants was balanced by a fall in the rate at which cholesterol was converted <jats:italic>via<\/jats:italic> cholesterol 24\u2010hydroxylase (Cyp46A1) to 24<jats:italic>S<\/jats:italic>\u2010hydroxycholesterol (24<jats:italic>S<\/jats:italic>\u2010OHC), the principal route through which cholesterol is ordinarily removed from the brain. Here, we show that while there were no genotypic differences in the concentrations in plasma and liver of three cholesterol precursors (lanosterol, lathosterol, and desmosterol), two plant sterols (sitosterol and campesterol), and two oxysterols (27\u2010hydroxycholesterol [27\u2010OHC] and 24<jats:italic>S<\/jats:italic>\u2010OHC), the brains of the <jats:italic>Mecp2<\/jats:italic><jats:sup>\n<jats:italic>\u2212\/y<\/jats:italic><\/jats:sup> mice had significantly lower concentrations of all three cholesterol precursors, campesterol, and both oxysterols, with the level of 24<jats:italic>S<\/jats:italic>\u2010OHC being ~20% less than in their <jats:italic>Mecp2<\/jats:italic><jats:sup>\n<jats:italic>+<\/jats:italic>\/<jats:italic>y<\/jats:italic><\/jats:sup> controls. Together, these data suggest that coordinated regulation of cholesterol synthesis and catabolism in the central nervous system is maintained in this model for RS. Furthermore, we speculate that the adaptive changes in these two pathways conceivably resulted from a shift in the permeability of the blood\u2013brain barrier as implied by the significantly lower campesterol and 27\u2010OHC concentrations in the brains of the <jats:italic>Mecp2<\/jats:italic><jats:sup><jats:italic>\u2212\/y<\/jats:italic><\/jats:sup> mice.<\/jats:p>","DOI":"10.1002\/lipd.12041","type":"journal-article","created":{"date-parts":[[2018,5,17]],"date-time":"2018-05-17T04:45:27Z","timestamp":1526532327000},"page":"363-373","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Identification of Correlative Shifts in Indices of Brain Cholesterol Metabolism in the C57BL6\/<i>Mecp2<\/i><sup><i>tm1.1Bird<\/i><\/sup> Mouse, a Model for Rett Syndrome"],"prefix":"10.1002","volume":"53","author":[{"given":"Dieter","family":"L\u00fctjohann","sequence":"first","affiliation":[{"name":"Institute for Clinical Chemistry 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