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

Stable Identifier
Reaction [transition]
Homo sapiens
cholesta-7,24-dien-3beta-ol + NADPH + H+ + O2 => cholesta-5,7,24-trien-3beta-ol + NADP+ + 2 H2O
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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

Balli, F, Andria, G, Rivasi, F, Dello Russo, A, Brunetti-Pierri, N, Ferrari, P, Ballabio, A, Annunziata, I, Corso, G, Rossi, M, Parenti, G

Am J Hum Genet 2002
12812989 Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency

Wassif, CA, Kovarova, M, Harris, G, Tsokos, M, Porter, FD, Grinberg, A, Hunter, AG, Krakowiak, PA, Yang, Y, Cozma, D, Kratz, L, Kelley, RI

Hum Mol Genet 2003
Catalyst Activity

C-5 sterol desaturase activity of SC5D [endoplasmic reticulum membrane]

Orthologous Events
Cross References
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