etx-ped-ch8-fig1

Figure 1 Adrenal steroidogenesis: Five enzymatic steps necessary for cortisol production are shown in numbers. 1= 20, 22 desmolase, 2= 17 hydroxylase (17-OH), 3=3ß-hydroxysteroid dehydrogenase (3ß HSD), 4=21 hydroxylase (21OHD), 5=11ß hydroxylase (11-OH) In the first step of adrenal stereoidgeneis, cholesterol enters mitochondria via a carrier protein called StAR. ACTH stimulates cholesterol cleavage, the rate limiting step of adrenal steroidogenesis.