Reactome | Cholesta-7,24-dien-3beta-ol is desaturated to form cholesta-5,7,24-trien-3beta-ol

Cholesta-7,24-dien-3beta-ol is desaturated to form cholesta-5,7,24-trien-3beta-ol

Stable Identifier

R-HSA-195664

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, endoplasmic reticulum membrane

Synonyms

5alpha-cholesta-7,24-dien-3beta-ol + NADPH + O2 + H(+) => 7-dehydrodesmosterol + NADP(+) + 2 H2O

ReviewStatus

5/5

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Cholesta-7,24-dien-3beta-ol is desaturated to form cholesta-5,7,24-trien-3beta-ol

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Cholesta-7,24-dien-3beta-ol, NADPH + H+, and O2 react to form cholesta-5,7,24-trien-3beta-ol, NADP+, and 2 H2O, catalyzed by SC5D. This reaction takes place in the endoplasmic reticulum. Its biochemical details are inferred from those of the reaction catalyzed by the purified rat protein (Kawata et al. 1985). The role of human SC5D in catalyzing this reaction in vivo is established from studies of patients in whom the enzyme is defective (Brunetti-Pierri et al. 2002; Krakowiak et al. 2003).

Literature References

PubMed ID	Title	Journal	Year
12189593	Lathosterolosis, a novel multiple-malformation/mental retardation syndrome due to deficiency of 3beta-hydroxysteroid-delta5-desaturase Brunetti-Pierri, N, Corso, G, Rossi, M, Ferrari, P, Balli, F, Rivasi, F, Annunziata, I, Ballabio, A, Dello Russo, A, Andria, G, Parenti, G	Am J Hum Genet	2002
12812989	Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency Krakowiak, PA, Wassif, CA, Kratz, L, Cozma, D, Kovarova, M, Harris, G, Grinberg, A, Yang, Y, Hunter, AG, Tsokos, M, Kelley, RI, Porter, FD	Hum Mol Genet	2003