SC5D desaturates LTHSOL to 7-dehydroCHOL
LTHSOL + 2 H+ + O2 + 2 CYP450:Fe2+ → 7-dehydroCHOL + 2 H2O + 2 CYP450:Fe3+
- Reactome
- R-HSA-6807053
- Compartment
- endoplasmic reticulum membrane · cytosol
- Rhea
- RHEA:46557
Inputs
- LTHSOLCHEBI:17168, C27H46O, endoplasmic reticulum membrane
- 2 H+CHEBI:15378, H, cytosol
- O2CHEBI:15379, O2, cytosol
- 2 CYP450:Fe2+R-HSA-8957354, endoplasmic reticulum membrane
Outputs
- 7-dehydroCHOLCHEBI:17759, C27H44O, endoplasmic reticulum membrane
- 2 H2OCHEBI:15377, H2O, cytosol
- 2 CYP450:Fe3+R-HSA-8957351, endoplasmic reticulum membrane
Catalysed by
- C-5 sterol desaturase activity of SC5D [endoplasmic reticulum membrane]UniProt O75845
Literature
- Lathosterolosis, a novel multiple-malformation/mental retardation syndrome due to deficiency of 3beta-hydroxysteroid-delta5-desaturase Am J Hum Genet, 2002
- Preputial gland tumor sterols. 3. A metabolic pathway from lanosterol to cholesterol J. Biol. Chem., 1960
- Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency Hum Mol Genet, 2003
- Flux analysis of cholesterol biosynthesis in vivo reveals multiple tissue and cell-type specific pathways Elife, 2015