What does 11 beta hydroxysteroid dehydrogenase do?
The enzyme 11-beta-hydroxysteroid dehydrogenase (EC 1.1. 1.146) converts active cortisol into inactive cortisone. The HSD11B2 gene (OMIM 614232) encodes the isoenzyme that is expressed in the kidney and which plays a particularly important role in blood pressure regulation.
What is the HSD enzyme?
Hydroxysteroid dehydrogenases (HSDs) are a group of alcohol oxidoreductases that catalyze the dehydrogenation of hydroxysteroids. These enzymes also catalyze the reverse reaction, acting as ketosteroid reductases (KSRs).
What action s are fulfilled by the enzyme 11 beta hydroxysteroid dehydrogenase?
11β-Hydroxysteroid dehydrogenase (HSD-11β or 11β-HSD) enzymes catalyze the conversion of inert 11 keto-products (cortisone) to active cortisol, or vice versa, thus regulating the access of glucocorticoids to the steroid receptors.
What is AME disease?
The syndrome of apparent mineralocorticoid excess (AME) is an autosomal recessive disorder characterized by hypertension, hypokalemia, low renin, and hypoaldosteronism. It is caused by deficiency of 11β-hydroxysteroid dehydrogenase, which results in a defect of the peripheral metabolism of cortisol to cortisone.
Why does 11 beta hydroxylase cause hypertension?
A buildup in the precursors used to form corticosterone increases salt retention, leading to hypertension in individuals with the classic form of CAH due to 11-beta-hydroxylase deficiency.
What is the hormone that burns fat?
Leptin: How This Fat Burning Hormone Works. We’ve all heard the term ‘fat-burning’, but how does it really happen in the body? Fat cells in the body release a hormone that signals to the brain that there is enough energy stored. This triggers your body to burn energy stored as fat.
What enzyme converts cortisol to cortisone?
In the kidney the enzyme 11β-hydroxysteroid dehydrogenase type 2 (11βHSD-2) converts cortisol into inactive cortisone, and so stops cortisol from binding to the mineralocorticoid receptors ( Walker 1994).
How do you treat AME?
The treatment of AME is primarily directed at the correction of hypokalemia and hypertension. Spironolactone, an MR receptor antagonist, is the medication of choice. Addition of potassium-sparing diuretics may be beneficial, but patients can become refractory to therapy.
How is 11 beta hydroxylase deficiency diagnosed?
Congenital Adrenal Hyperplasia Caused by 11Beta-Hydroxylase Deficiency. and increased production of adrenal androgens, leading to virilization. Diagnosis is by measurement of cortisol, its precursors, and adrenal androgens and sometimes by measuring 11-deoxycortisol after adrenocorticotropic hormone administration.