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Aldosterone synthase, also called steroid 18-hydroxylase, corticosterone 18-monooxygenase or P450C18, is a steroid hydroxylase cytochrome P450 enzyme involved in the biosynthesis of the mineralocorticoid aldosterone and other steroids. The enzyme catalyzes sequential hydroxylations of the steroid angular methyl group at C18 after initial 11β ...
Hypoaldosteronism. Hypoaldosteronism is an endocrinological disorder characterized by decreased levels of the hormone aldosterone. Similarly, isolated hypoaldosteronism is the condition of having lowered aldosterone without corresponding changes in cortisol. [1] (. The two hormones are both produced by the adrenals.)
In adrenal insufficiency, there is a deficiency in cortisol production which may be accompanied by a deficiency in aldosterone production (predominantly in primary adrenal insufficiency). [2] [3] Depending on the cause and type of adrenal insufficiency, the mechanism of the disease differs. Generally, the symptoms manifest through the systemic ...
These enzymes are nearly identical (they share 11β-hydroxylation and 18-hydroxylation functions), but aldosterone synthase is also able to perform an 18-oxidation. Moreover, aldosterone synthase is found within the zona glomerulosa at the outer edge of the adrenal cortex; 11β-hydroxylase is found in the zona glomerulosa and zona fasciculata.
The genes encoding aldosterone synthase and 11β-hydroxylase are 95% identical and are close together on chromosome 8. In individuals with GRA, there is unequal crossing over so that the 5' regulatory region of the 11-hydroxylase gene is fused to the coding region of the aldosterone synthase. [citation needed]
Hypoaldosteronism is a clinical condition marked by either an aldosterone deficiency or impaired tissue-level action of the hormone. Angiotensin I to Angiotensin II conversion, adrenal aldosterone synthesis and secretion, abnormal target tissue response to aldosterone, and renal renin production and secretion are all potential causes of the ...
Glucocorticoids. [2] Adrenocorticotropic hormone deficiency is a rare disorder characterized by secondary adrenal insufficiency with minimal or no cortisol production and normal pituitary hormone secretion apart from ACTH. [3] ACTH deficiency may be congenital or acquired, and its symptoms are clinically similar to those of glucocorticoid ...
It may be increased in 17α-hydroxylase deficiency, [4] in aldosterone synthase deficiency, [5] in primary aldosteronism, and may also indicate a histologic variant of the aldosteronoma. [4] Excessive secretion of 18-OH-DOC can cause mineralocorticoid excess syndrome, although these cases are very rare.