What is the function of desmolase?
A desmolase is any of various enzymes that catalyze the formation or destruction of carbon-carbon bonds within a molecule. These enzymes play a significant role in cellular respiration and in fermentation. Desmolases are involved in steroidogenesis.
Where is desmolase found?
Side chain cleavage enzyme (also known as cholesterol desmolase) is a cytochrome P450 enzyme encoded by the CYP11A1 gene located at chromosome 15q23-q24. This enzyme converts cholesterol to pregnenolone, is essential to steroidogenesis, and plays a crucial role in placental progesterone synthesis.
What is adrenal steroidogenesis?
Adrenal steroidogenesis is a dynamic process, reliant on de novo synthesis from cholesterol, under the stimulation of ACTH and other regulators. The syntheses of mineralocorticoids, glucocorticoids and adrenal androgens occur in separate adrenal cortical zones, each expressing specific enzymes.
What is the meaning of steroidogenesis?
Steroidogenesis is the multistep process for biosynthesis of steroid hormones from cholesterol. Primary organs for steroidogenesis in the male are the pair of testes and adrenal glands.
What is cholesterol Desmolase deficiency?
Conversion of cholesterol to pregnenolone is mediated by the cholesterol side-chain cleavage (SCC) enzyme, P450scc. Deficient SCC activity causes congenital lipoid adrenal hyperplasia (also known as 20,22 desmolase deficiency), a potentially lethal defect in the synthesis of all steroid hormones.
What is metyrapone test?
The metyrapone stimulation test is based upon the principle that decreasing serum cortisol concentrations normally produces an increase in corticotropin (ACTH) secretion due to a decrease in glucocorticoid negative feedback. The test is performed primarily to detect partial defects in pituitary ACTH secretion.
What is gonadal steroidogenesis?
Gonadal steroidogenesis is pivotal to synchronize various reproductive stages including sexual development, growth and maturation. In all vertebrates including teleost, steroidogenesis is triggered by the mobilization of cholesterol by steroidogenic acute regulatory protein from outer to inner mitochondrial membrane.
What is lipoid CAH?
Lipoid congenital adrenal hyperplasia (lipoid CAH), a rare disorder of steroid biosynthesis, is the most severe form of congenital adrenal hyperplasia (CAH).In this disorder the synthesis of all adrenal and gonadal steroid hormones is impaired due to a molecular defect in the steroidogenic acute regulatory protein.