Reactome | Zymosterol is isomerized to cholesta-7,24-dien-3beta-ol

Zymosterol is isomerized to cholesta-7,24-dien-3beta-ol

Stable Identifier

R-HSA-195690

Type

Reaction [transition]

Species

Homo sapiens

Compartment

endoplasmic reticulum membrane

Synonyms

Zymosterol => cholesta-7,24-dien-3beta-ol

ReviewStatus

5/5

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Zymosterol is isomerized to cholesta-7,24-dien-3beta-ol

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Isomerization of zymosterol to cholesta-7,24-dien-3beta-ol is catalyzed by EBP in the endoplasmic reticulum. The biochemical details of the reaction have been established through studies of purified rat EBP; the role of the human enzyme has been established through studies of patients deficient in it (Derry et al. 1999; Braverman et al. 1999).

Literature References

PubMed ID	Title	Journal	Year
10391219	Mutations in the gene encoding 3 beta-hydroxysteroid-delta 8, delta 7-isomerase cause X-linked dominant Conradi-Hunermann syndrome Wilcox, WR, Obie, C, Lin, P, Valle, D, Moser, A, Moebius, FF, Rimoin, DL, Glossmann, H, Braverman, N, Kratz, L, Kelley, RI, Smith, M	Nat Genet	1999
10391218	Mutations in a delta 8-delta 7 sterol isomerase in the tattered mouse and X-linked dominant chondrodysplasia punctata. jderry@immunex.com. Boyd, Y, Herman, GE, Gormally, E, Zhao, W, Means, GD, Derry, JM, Kelley, RI, Meindl, A	Nat Genet	1999