Sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating)

Sterol - 4α -carboxylate -3- dehydrogenase is an enzyme that several parallel reactions in the biosynthesis of cholesterol which catalyzes a carboxyl group cleaved in the 4 position and a hydroxy group in each case is reduced to the 3-position to the ketone. The enzyme is found in animals and fungi. In humans it is produced in particular in brain, heart, liver, lungs, kidneys, skin, and placenta. Mutations in NSDHL gene are responsible for the CHILD syndrome, a rare genetic disease.

Catalyzed reaction

NADP ⇒ CO2 NADPH / H

4alpha Methylzymosterin - 4-carboxylate is converted to 3-keto -4- methylzymosterin. Substrates may differ in the degree of methylation.

Human Genome Organisation
748813
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