Sterol Δ24 reductase
| Sterol Δ24 reductase | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 494 amino acids | |
| Secondary to quaternary structure | single-pass membrane protein (ER, Golgi) | |
| Cofactor | FAD | |
| Identifier | ||
| Gene name | DHCR24 | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 1.3.1.- , oxidoreductase | |
| Response type | Hydrogenation of a double bond | |
| Substrate | 24-double bond sterol + NADPH / H + | |
| Products | 24-single bond sterol + NADP + | |
| Occurrence | ||
| Parent taxon | Eukaryotes | |
| Orthologue | ||
| human | House mouse | |
| Entrez | 1718 | 74754 |
| Ensemble | ENSG00000116133 | ENSMUSG00000034926 |
| UniProt | Q15392 | Q8VCH6 |
| Refseq (mRNA) | NM_014762 | NM_053272 |
| Refseq (protein) | NP_055577 | NP_444502 |
| Gene locus | Chr 1: 54.85 - 54.89 Mb | Chr 4: 106.56 - 106.59 Mb |
| PubMed search | 1718 |
74754
|
The sterol Δ24 reductase (also: 24-dehydrocholesterol reductase), the enzyme that the hydrogenation of sterols at the 24-double bond catalyzed . The reaction takes place in all eukaryotes during cholesterol biosynthesis . The enzyme is localized in the membrane of the Golgi apparatus and the endoplasmic reticulum . In humans, it is mainly produced in the brain and adrenal glands , but also in the liver , lungs , spleen , prostate and spine . Mutations in DHCR24 - gene are responsible for rare inherited disorder Desmosterolose responsible.
Catalyzed reaction
+ NADP H / H + ⇔ 
+ NADP +
5α-Cholesta-7,24-dien-3β-ol is hydrogenated to lathosterol.
+ NADP H / H + ⇔ 
+ NADP +
Desmosterin is hydrogenated to cholesterol.
Individual evidence
Web links
Wikibooks: Biochemistry and Pathobiochemistry: Cholesterol Biosynthesis - Learning and Teaching Materials
- D'Eustachio / reactome: Reduction of desmosterol to cholesterol
- OrphaNet: Desmosterolosis