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hydroxylase |
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hydroxylase /hy·drox·y·lase/ (hi-drok´sĭ-lās) any of a group of enzymes that catalyze the formation of a hydroxyl group on a substrate by incorporation of one atom (monooxygenases) or two atoms (dioxygenases) of oxygen from O2. 11β-hydroxylase an enzyme that catalyzes the hydroxylation of steroids at the 11 position, a step in the synthesis of steroid hormones; deficiency causes a form of congenital adrenal hyperplasia. 17α-hydroxylase an enzyme that catalyzes the oxidation of steroids at the 17 position, steps in the synthesis of steroid hormones; deficiency causes a form of congenital adrenal hyperplasia and if it occurs during gestation can cause male pseudohermaphroditism. 18-hydroxylase an enzyme that catalyzes several steps in the biosynthesis of aldosterone from corticosteroids; deficiency causes salt wasting. 21-hydroxylase an enzyme that catalyzes the hydroxylation of steroids at the 21 position, a step in the synthesis of steroid hormones; deficiency impairs the ability to produce all glucocorticoids and causes a form of congenital adrenal hyperplasia.
hydroxylase any enzyme that brings about the coupled oxidation of two donors, with incorporation of oxygen into one of them. 21-hydroxylase a partial deficiency of the enzyme, in Pomeranian dogs, is thought to cause a dermatosis; histopathological lesions are of cutaneous atrophy; clinical signs include bilateral alopecia of the trunk, caudal thighs, ventral neck. How to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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| ? Mentioned in | ? References in periodicals archive | |
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The most common type of
CAH is 21-hydroxylase deficiency, within which three severity subtypes
are distinguished: Saltwasters (SW), the most severe subtype, in which
the enzyme deficiency causes deficiencies of both glucocorticoid
(glucose-regulating) and mineralocorticoid (sodium-regulating) hormones;
simple virilizers (SV), with moderate deficiencies of glucocorticoids;
and the mildest subtype, non-classical (NC), late-onset CAH. I focused on the most
prevalent intersex syndrome, classical (prenatal-onset) congenital
adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, and
summarized recent psychological research in children and adults with
this syndrome, complemented by studies on intersex syndromes in 46, XY
individuals. Amniotic fluid concentrations of
delta-5 and delta-4 steroids in fetuses with congenital adrenal
hyperplasia due to 21-hydroxylase deficiency and in anencephalic
fetuses. |
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