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Loss\u2010of\u2010function mutations in\n                    <jats:italic>LIPA<\/jats:italic>\n                    result in cholesteryl ester storage disease (CESD) or Wolman disease when mutations cause complete loss of LAL activity. Although the phenotype of a mouse CESD model has been extensively characterized, there has not been a focus on the brain at different stages of disease progression. In the current studies, whole\u2010brain mass and the concentrations of cholesterol in both the esterified (EC) and unesterified (UC) fractions were measured in\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    and matching\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>+\/+<\/jats:italic>\n                    <\/jats:sup>\n                    mice (FVB\u2010N strain) at ages ranging from 14 up to 280\u2009days after birth. Compared to\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>+\/+<\/jats:italic>\n                    <\/jats:sup>\n                    controls at 50, 68\u201376, 140\u2013142, and 230\u2013280\u2009days of age,\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice had brain weights that averaged approximately 6%, 7%, 18%, and 20% less, respectively. Brain EC levels were higher in the\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice at every age, being elevated 27\u2010fold at 230\u2013280\u2009days. Brain UC concentrations did not show a genotypic difference at any age. The elevated brain EC levels in the\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice did not reflect EC in residual blood. An mRNA expression analysis for an array of genes involved in the synthesis, catabolism, storage, and transport of cholesterol in the brains of 141\u2010day old mice did not detect any genotypic differences although the relative mRNA levels for several markers of inflammation were moderately elevated in the\n                    <jats:italic>Lal<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice. The possible sites of EC accretion in the central nervous system are discussed.\n                  <\/jats:p>","DOI":"10.1002\/lipd.12325","type":"journal-article","created":{"date-parts":[[2021,10,9]],"date-time":"2021-10-09T09:05:44Z","timestamp":1633770344000},"page":"3-16","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Molecular markers of brain cholesterol homeostasis are unchanged despite a smaller brain mass in a mouse model of cholesteryl ester storage disease"],"prefix":"10.1002","volume":"57","author":[{"given":"Amal A.","family":"Aqul","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charina M.","family":"Ramirez","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Texas Southwestern Medical Center  Dallas Texas 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