Sterol-4 α -carboxylate-3-dehydrogenase

Sterol 4α-carboxylate-3-dehydrogenase
Properties of human protein
Mass / length primary structure	373 amino acids
Secondary to quaternary structure	Membrane protein
Identifier
Gene name	NSDHL
External IDs	OMIM : 300275 ; UniProt : Q15738 ;
Enzyme classification
EC, category	1.1.1.170 , oxidoreductase
Response type	Dehydration, decarboxylation
Substrate	(4-methyl-) zymosterol-4-carboxylate + NADP +
Products	3-keto- (4-methyl-) zymosterol + CO 2 + NADPH / H +
Occurrence
Parent taxon	Eukaryotes
	Orthologue

The sterol-4 α -carboxylate-3-dehydrogenase is an enzyme that catalyzes several parallel reactions in the cholesterol biosynthesis , whereby in each case a carboxy group in the 4-position is split off and a hydroxyl group in the 3-position is reduced to the ketone . The enzyme can be found in animals and fungi. In humans, it is especially produced in the brain , heart , liver , lungs , kidneys , skin and placenta . Mutations in NSDHL - gene are for the CHILD syndrome responsible for a rare hereditary disease.

Catalyzed reaction

+ NADP ⁺ ⇒ + CO ₂ + NADPH / H ⁺

4 α -methylzymosterol-4-carboxylate is converted to 3-keto-4-methylzymosterol. Substrates can differ in the degree of methylation.

Wikibooks: Biochemistry and Pathobiochemistry: Cholesterol Biosynthesis - Learning and Teaching Materials