What is the function of desmolase?

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.

Where is cholesterol 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 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.

Which enzyme required for cholesterol convert to Pregenolone?

Within mitochondria, cholesterol is converted to pregnenolone by an enzyme in the inner membrane called CYP11A1.

Where is pregnenolone produced in the body?

Pregnenolone is a hormone naturally produced in the body by the adrenal gland. Pregnenolone is also made from cholesterol, and is the starting material in the production of testosterone, progesterone, cortisol, estrogen and other hormones.

What is testicular steroidogenesis?

Testicular steroidogenesis is a fine-regulated process, and its main product, testosterone (T), has a crucial role in fetal development and maturation and in adulthood for the maintenance of secondary sexual function and spermatogenesis.

How does desmolase convert cholesterol to pregnenolone?

Cholesterol desmolase (side-chain cleavage enzyme, P450scc, CYP11A) converts cholesterol to pregnenolone. This is accomplished through three oxidations: 22Rr and 20Rrhydroxylations and oxidative cleavage of the 20,22 carbon–carbon bond. These reactions require a total of three O 2 molecules and six electrons.

What is the name of the enzyme that cleavages cholesterol?

View/Edit Mouse. Cholesterol side-chain cleavage enzyme is commonly referred to as P450scc, where “scc” is an acronym for side-chain cleavage. P450scc is a mitochondrial enzyme that catalyzes conversion of cholesterol to pregnenolone.

Which is cholesterol monooxygenase cleaves the side chain?

P450scc can also cleave the side-chain of other hydroxysterols such as 7-dehydrocholesterol to form 7-dehydropregnenolone, and hydroxylate vitamin D3 to form 20-hydroxyvitamin D3 and 20,22-dihydroxyvitamin D3 ( Guryev et al., 2003 ).

Where is P450scc located in the mitochondrial membrane?

P450scc is associated with the inner mitochondrial membrane, facing the interior (matrix). Adrenodoxin and adrenodoxin reductase are soluble peripheral membrane proteins located inside the mitochondrial matrix that appear to associate with each other primarily through electrostatic interactions.