TY - JOUR
T1 - Disorders of steroid 11β-hydroxylase isozymes
AU - White, Perrin C.
AU - Pascoe, Leigh
PY - 1992/8
Y1 - 1992/8
N2 - Steroid 11 β-hydroxylase activity in the adrenal cortex is required for the synthesis of the major glucocorticoids and mineralocorticoids, but different isozymes mediate this conversion in the zona fasciculata, where cortisol is produced, and the zona glomerulosa, the site of aldosterone synthesis. The isozyme in the latter zone also has 18-hydroxylase and 18-oxidase activities that are required for aldosterone synthesis. Mutations in the genes encoding these isozymes respectively result in defective synthesis of cortisol and aldosterone. Recombinations between the two genes that alter the regulation of the isozyme responsible for aldosterone synthesis cause an inherited form of hypertension, glucocorticoid-suppressible hyperaldosteronism.
AB - Steroid 11 β-hydroxylase activity in the adrenal cortex is required for the synthesis of the major glucocorticoids and mineralocorticoids, but different isozymes mediate this conversion in the zona fasciculata, where cortisol is produced, and the zona glomerulosa, the site of aldosterone synthesis. The isozyme in the latter zone also has 18-hydroxylase and 18-oxidase activities that are required for aldosterone synthesis. Mutations in the genes encoding these isozymes respectively result in defective synthesis of cortisol and aldosterone. Recombinations between the two genes that alter the regulation of the isozyme responsible for aldosterone synthesis cause an inherited form of hypertension, glucocorticoid-suppressible hyperaldosteronism.
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U2 - 10.1016/1043-2760(92)90033-W
DO - 10.1016/1043-2760(92)90033-W
M3 - Review article
C2 - 18407105
AN - SCOPUS:0026644277
SN - 1043-2760
VL - 3
SP - 229
EP - 234
JO - Trends in Endocrinology and Metabolism
JF - Trends in Endocrinology and Metabolism
IS - 6
ER -