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The response of all ACTH\u2010dependent steroids to Synacthen was severely impaired in all patients, despite normal basal levels. It can be concluded that the biochemical defect in adrenal steroidogenesis causing the inadequate response to ACTH injection is located proximal to progesterone.<\/jats:p><jats:p>We propose that the lack of responsiveness to ACTH is secondary to an abnormality of ACTH receptors on the adrenocortical cell. The extremely low levels of non\u2010specific lipid transfer protein may be a contributing factor.<\/jats:p>","DOI":"10.1007\/bf01802037","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T23:58:04Z","timestamp":1119916684000},"page":"423-428","source":"Crossref","is-referenced-by-count":9,"title":["Further analysis of the disturbed adrenocortical function in the cerebro\u2010hepato\u2010renal syndrome of zellweger"],"prefix":"10.1002","volume":"12","author":[{"given":"L.","family":"Govaerts","sequence":"first","affiliation":[{"name":"Department of Human Genetics University of Nijmegen  Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W. 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