MSMO1 oxidizes 4a-me-5a-cholest-8-en-3b-ol to 4a-carboxy-5a-cholest-8-ene-3b-ol

Stable Identifier
R-HSA-9947207
Type
Reaction [transition]
Species
Homo sapiens
Compartment
endoplasmic reticulum membrane, cytosol
Synonyms
4alpha-methyl-5alpha-cholest-8-en-3beta-ol + 6 Fe(II)-(cytochrome b5) + 3 O2 + 5 H(+) => 4alpha-carboxy-5alpha-cholest-8-ene-3beta-ol + 6 Fe(III)-(cytochrome b5) + 4 H2O
ReviewStatus
5/5
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MSMO1 oxidizes 4a-me-5a-cholest-8-en-3b-ol to 4a-carboxy-5a-cholest-8-ene-3b-ol
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MSMO1 (Methylsterol monooxygenase 1), associated with the endoplasmic reticulum membrane, oxidizes 4a-me-5a-cholest-8-en-3b-ol to 4a-carboxy-5a-cholest-8-en-3b-ol. Evidence for this function of human MSMO1 protein is indirect, coming from studies of steroid synthesis intermediates accumulated in patients with defects in the enzyme (He et al. 2011).
Literature References
PubMed ID Title Journal Year
21285510 Mutations in the human SC4MOL gene encoding a methyl sterol oxidase cause psoriasiform dermatitis, microcephaly, and developmental delay

He, M, Kratz, LE, Michel, JJ, Vallejo, AN, Ferris, L, Kelley, RI, Hoover, JJ, Jukic, D, Gibson, KM, Wolfe, LA, Ramachandran, D, Zwick, ME, Vockley, J

J Clin Invest 2011
Participants
Input
Output
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as an event of
Catalyst Activity

C-4 methylsterol oxidase activity of MSMO1 [endoplasmic reticulum membrane]

Physical Entity
MSMO1 [endoplasmic reticulum membrane] (Homo sapiens)
Activity
C-4 methylsterol oxidase activity (GO:0000254)
Cross References
RHEA
Authored
D'Eustachio, P  (2025-05-02)
Reviewed
Hill, DP  (2025-05-30)
Created
D'Eustachio, P  (2025-05-02)
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