{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T05:42:00Z","timestamp":1790487720228,"version":"4.1.0"},"reference-count":48,"publisher":"The Endocrine Society","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,6,1]]},"abstract":"<jats:p>Steroidogenic acute regulatory protein (StAR) facilitates cholesterol transfer into the inner mitochondrial membrane in the acute phase of steroidogenesis. Mice lacking StAR (Star\u2212\/\u2212) share phenotypes with human individuals having congenital lipoid adrenal hyperplasia including compromised production of steroid hormones and florid accumulation of cholesterol esters in adrenal glands and gonads. To define a specific pattern of molecular changes with StAR deficiency, we performed transcriptome analysis of adrenal cells selectively isolated by fluorescent-activated cell sorting at embryonic d 17.5 or 18.5 in seven wild-type (Star+\/+) or four Star\u2212\/\u2212 mice having the transgene targeting the enhanced green fluorescent protein to cell lineages that express StAR. A gene expression profile was obtained by whole-mouse genome microarray and confirmed by quantitative real-time PCR, identifying 1206 and 767 significantly up-regulated and down-regulated genes, respectively, in Star\u2212\/\u2212 mice compared with Star+\/+ mice (fold difference \u2265 2 and P value &amp;lt; 0.05 with false discovery rate &amp;lt; 0.2). In Star\u2212\/\u2212 mice, expression levels of genes involved in cholesterol efflux and the inflammatory response were significantly up-regulated, whereas those related to steroid hormone biosynthesis or cholesterol biosynthesis and influx were not significantly changed. Immunoreactive Iba1 or F4\/80 (macrophage marker) in adrenal glands of Star\u2212\/\u2212 mice was detected not only in an increased number of resident macrophages but also in most adrenocortical cells. These findings expand our understanding of the pathophysiology of adrenal glands with the disruption of StAR and propose a reciprocal interaction between adrenocortical cells and resident macrophages inside adrenal glands of Star\u2212\/\u2212 mice.<\/jats:p>","DOI":"10.1210\/en.2011-1627","type":"journal-article","created":{"date-parts":[[2012,4,24]],"date-time":"2012-04-24T05:22:57Z","timestamp":1335244977000},"page":"2714-2723","source":"Crossref","is-referenced-by-count":20,"title":["A Genome-Wide Expression Profile of Adrenocortical Cells in Knockout Mice Lacking Steroidogenic Acute Regulatory Protein"],"prefix":"10.1210","volume":"153","author":[{"given":"Tomohiro","family":"Ishii","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Physiology, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshikatsu","family":"Mitsui","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Physiology, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sadafumi","family":"Suzuki","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Physiology, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yumi","family":"Matsuzaki","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Physiology, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonobu","family":"Hasegawa","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Physiology, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2012,4,23]]},"reference":[{"key":"2020071612164190500_B1","doi-asserted-by":"crossref","first-page":"7899","DOI":"10.1074\/jbc.272.12.7899","article-title":"Submitochondrial distribution of three key steroidogenic proteins (steroidogenic acute regulatory protein and cytochrome p450scc and 3\u03b2-hydroxysteroid dehydrogenase isomerase enzymes) upon stimulation by intracellular calcium in adrenal glomerulosa cells.","volume":"272","author":"Cherradi","year":"1997","journal-title":"J Biol Chem"},{"key":"2020071612164190500_B2","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/S0303-7207(01)00423-3","article-title":"Elements involved in the regulation of the StAR gene.","volume":"177","author":"Stocco","year":"2001","journal-title":"Mol Cell Endocrinol"},{"key":"2020071612164190500_B3","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1146\/annurev.physiol.63.1.193","article-title":"StAR protein and the regulation of steroid hormone biosynthesis.","volume":"63","author":"Stocco","year":"2001","journal-title":"Annu Rev Physiol"},{"key":"2020071612164190500_B4","doi-asserted-by":"crossref","first-page":"46583","DOI":"10.1074\/jbc.M107815200","article-title":"Mitochondrial processing of newly synthesized steroidogenic acute regulatory protein (StAR), but not total StAR, mediates cholesterol transfer to cytochrome P450 side chain cleavage enzyme in adrenal cells.","volume":"276","author":"Artemenko","year":"2001","journal-title":"J Biol Chem"},{"key":"2020071612164190500_B5","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1172\/JCI0216771","article-title":"High-flux mitochondrial cholesterol trafficking, a specialized function of the adrenal cortex.","volume":"110","author":"Jefcoate","year":"2002","journal-title":"J Clin Invest"},{"key":"2020071612164190500_B6","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1038\/417087a","article-title":"Rapid regulation of steroidogenesis by mitochondrial protein import.","volume":"417","author":"Bose","year":"2002","journal-title":"Nature"},{"key":"2020071612164190500_B7","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1210\/er.2010-0013","article-title":"The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders.","volume":"32","author":"Miller","year":"2011","journal-title":"Endocr Rev"},{"key":"2020071612164190500_B8","doi-asserted-by":"crossref","first-page":"1870","DOI":"10.1056\/NEJM199612193352503","article-title":"The pathophysiology and genetics of congenital lipoid adrenal hyperplasia. 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