An ACTH Receptor on Human Mononuclear Leukocytes

THE inaccessibility of many tissues makes routine investigation for receptor defects impossible. ACTH insensitivity syndrome, first described by Shepard et al.¹ in 1959 and now reported in more than 63 patients,^{1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19} is an example of this problem. Defects in adrenal ACTH receptors have been postulated because of high circulating ACTH levels and the ability of the adrenal-cortex cells to achieve steroidogenesis through an elevation of cyclic AMP.^{1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19} In the majority of cases, the clinical presentation includes hyperpigmentation, hypoglycemia, and extremely low glucocorticoid levels with normal mineralocorticoid concentrations.^{2 3 4 5 6 7 8 9 10 11 12 13 14 15 16} Differences between cases suggest that the syndrome may have multiple causes. Adrenal cells…