SC5D desaturates LTHSOL to 7-dehydroCHOL

Stable Identifier
R-HSA-6807053
Type
Reaction [transition]
Species
Homo sapiens
Compartment
endoplasmic reticulum membrane, cytosol
Synonyms
lathosterol + 2 Fe(II)-(cytochrome b5) + O2 + 2 H(+) => 7-dehydrocholesterol + 2 Fe(III)-(cytochrome b5) + 2 H2O
ReviewStatus
5/5
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SC5D desaturates LTHSOL to 7-dehydroCHOL
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SC5D (lathosterol oxidase) associated with the endoplasmic reticulum desaturates LTHSOL (lathosterol) to 7-dehydroCHOL (7-dehydrocholesterol) (Brunetti-Pierri et al. 2002; Kandutsch & Russell 1960; Krakowiek et al. 2003; Mitsche et al. 2015).
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
14404284 Preputial gland tumor sterols. 3. A metabolic pathway from lanosterol to cholesterol

Russell, AE, Kandutsch, AA

J. Biol. Chem. 1960
26114596 Flux analysis of cholesterol biosynthesis in vivo reveals multiple tissue and cell-type specific pathways

Hobbs, HH, Mitsche, MA, Cohen, JC, McDonald, JG

Elife 2015
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
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Output
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Catalyst Activity

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

Physical Entity
SC5D [endoplasmic reticulum membrane] (Homo sapiens)
Activity
C-5 sterol desaturase activity (GO:0000248)
Cross References
RHEA
Authored
D'Eustachio, P  (2015-11-02)
Reviewed
Jassal, B  (2015-11-02)
Hill, DP  (2025-05-30)
Created
D'Eustachio, P  (2015-10-29)
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