Figure 416

Normal and chimeric aldosterone synthase in glucocorticoid-remedial aldosteronism (GRA). A, Normal relationship between the stimuli and site of adrenal cortical steroid production. Aldosterone synthase normally responds to angiotensin II (AII) in the zona glomerulosa, resulting in aldosterone synthesis and release (see Figs. 4-2 and 4-3). B, In GRA, a chimeric aldosterone synthase gene results from a mutation, which stimulates production of aldosterone and other steroids from the zona glomerulosa under the control of adrenocorticotropic hormone (ACTH) (Fig. 4-17). Thus, when ACTH production is suppressed by steroid administration, aldos-terone production is reduced.

11-OHase

11-OHase

Aldosterone synthase Chimeric gene

11-OHase

Aldosterone synthase Chimeric gene

11-OHase

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