21-Hydroxylase

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21-Hydroxylase (pronounced as twenty-one hydroxylase) is an enzyme that plays a crucial role in the production of mineralocorticoids and glucocorticoids, which are types of hormones produced by the adrenal glands.

Etymology

The term "21-Hydroxylase" is derived from its function. The enzyme hydroxylates the 21st carbon atom in the steroid molecule during the biosynthesis of corticosteroids.

Function

21-Hydroxylase is involved in the biosynthesis of corticosteroids, specifically mineralocorticoids and glucocorticoids. It catalyzes the conversion of progesterone to deoxycorticosterone and 17α-hydroxyprogesterone to 11-deoxycortisol.

Clinical Significance

Deficiency of 21-hydroxylase is the most common cause of Congenital Adrenal Hyperplasia (CAH). This condition is characterized by an overproduction of androgens, leading to virilization and ambiguous genitalia in female newborns. In severe cases, it can lead to life-threatening adrenal crisis due to a lack of cortisol and aldosterone.

Related Terms

  • CYP21A2: The gene that encodes the 21-hydroxylase enzyme.
  • Steroidogenesis: The biological process by which steroids are generated from cholesterol.
  • Adrenal Gland: The gland where 21-hydroxylase is primarily located and functions.

See Also

External links

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