11-Deoxycortisol – 11-Deoxycortisol (Compound S) is useful in diagnosing patients with 11-beta-hydroxylase deficiency (second leading cause of congenital adrenal hyperplasia) and primary (adrenal failure) or secondary (hypothalamic-pituitary ACTH deficiency) adrenal insufficiency.
Is Deoxycortisol a mineralocorticoid?
We show here that 11-deoxycortisol functioned as both a glucocorticoid and a mineralocorticoid in the lamprey. Our findings indicate that a complex and highly specific corticosteroid signaling pathway evolved at least 500 million years ago with the arrival of the earliest vertebrate.
What does low 11-deoxycortisol mean?
Clinical significance As a result, the level of 11-deoxycortisol is measured to diagnose impaired cortisol synthesis, to find out the enzyme deficiency that causes impairment along the pathway to cortisol, and to differentiate adrenal disorders. In 21-hydroxylase deficiency, 11-deoxycortisol levels are low.
What converts 11-deoxycortisol to cortisol?
In the human adrenal, the mitochondrial cytochrome P450 enzyme CYP11B1 catalyses the conversion of 11-deoxycortisol to cortisol, a key reaction in cortisol biosynthesis that in addition is of fundamental interest for the technical synthesis of glucocorticoids.
How is 11-deoxycortisol synthesized?
11-Deoxycortisol is synthesized from 17α-hydroxyprogesterone by 21-hydroxylase and is converted to cortisol by 11β-hydroxylase. 11-Deoxycortisol in mammals has limited biological activity and mainly acts as metabolic intermediate within the glucocorticoid pathway, leading to cortisol.
What does a high 11-deoxycortisol level mean?
As a result, the level of 11-deoxycortisol is measured to diagnose impaired cortisol synthesis, to find out the enzyme deficiency that causes impairment along the pathway to cortisol, and to differentiate adrenal disorders.
What is 21 deoxycortisol used for?
21-deoxycortisol is a deoxycortisol that is 17xi-pregn-4-ene-3,20-dione substituted by a beta-hydroxy group at position 11 and an alpha-hydroxy group at position 17. It is a marker of virilizing adrenal hyperplasia caused by 21-hydroxylase deficiency. It has a role as a human blood serum metabolite and a mouse metabolite.
What is the role of 11-deoxycortisol in metyrapone testing?
Combining 11-deoxycortisol measurements with ACTH measurements during metyrapone testing further enhances the performance of the test. Impairment of any component of the HPA-axis results in a subnormal rise in 11-deoxycortisol levels.
