Abstract
Cortisol is “deactivated” by its conversion to cortisone through the actions of 11β-hydroxysteroid dehydrogenase (11β-HSD). 11β-HSD occurs as two distinct isoforms, namely, 11β-HSD1 that converts cortisone to cortisol and 11β-HSD2 which metabolises cortisol to cortisone. Deficiency states affecting both enzymes have been described and are characterised biochemically by an abnormal ratio of cortisol to cortisone metabolites in a urine steroid profile. The conditions have markedly different phenotypes: 11β-HSD2 is similar to Cushing’s syndrome and 11β-HSD1 characterised by androgen excess.
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Further Reading
Wider Role of 11β-HSD1
Cooper MS, Stewart PM. 11β-Hydroxysteroid dehydrogenase type 1 and its role in the hypothalamic-pituitary-adrenal axis, metabolic syndrome and inflammation. J Clin Endocrinol Metab. 2009;94:4645–54.
Bailey MA. 11β-Hydroxysteroid dehydrogenases and hypertension in the metabolic syndrome. Curr Hypertens Rep. 2017;19:100.
Update on Genetics and Biochemistry of 11β-HSD2
Yau M, Haider S, Khattab A, et al. Clinical, genetic, and structural basis of apparent mineralocorticoid excess due to 11β-hydroxysteroid dehydrogenase type 2 deficiency. Proc Natl Acad Sci U S A. 2017;114(52):E11248–56.
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Woodward, G.M. (2019). 11β-Hydroxysteroid Dehydrogenase Deficiency. In: Rumsby, G., Woodward, G. (eds) Disorders of Steroidogenesis. Springer, Cham. https://doi.org/10.1007/978-3-319-96364-8_12
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DOI: https://doi.org/10.1007/978-3-319-96364-8_12
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