Sterol delta 8/7 isomerase
| 3-β-hydroxysteroid Δ8,7 isomerase | ||
|---|---|---|
| other names |
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| Properties of human protein | ||
| Mass / length primary structure | 26.4 kilodaltons / 230 amino acids | |
| Secondary to quaternary structure | multipass membrane protein (ER) | |
| Identifier | ||
| Gene name | EBP | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 5.3.3.5 , isomerase | |
| Response type | Rearrangement | |
| Substrate | Delta (8) sterol | |
| Products | Delta (7) sterol | |
| Occurrence | ||
| Parent taxon | Eukaryotes | |
| Orthologue | ||
| human | House mouse | |
| Entrez | 10682 | 13595 |
| Ensemble | ENSMUSG00000031168 | |
| UniProt | Q15125 | P70245 |
| Refseq (mRNA) | NM_006579 | NM_007898 |
| Refseq (protein) | NP_006570 | NP_031924 |
| Gene locus | Chr X: 48.52 - 48.53 Mb | Chr X: 8.19 - 8.19 Mb |
| PubMed search | 10682 |
13595
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The sterol Δ8 / 7-isomerase (also: emopamil binding protein (EBP) ) is the enzyme , the sterol derivatives within the rearrangement of the located in the 8-position double bond in the 7-position catalyzed . This reaction takes place in all eukaryotes within the framework of cholesterol biosynthesis . It is a transmembrane protein that is located on the endoplasmic reticulum . Mutations in EBP - gene of humans are the cause of a type of chondrodysplasia punctata , called Conradi-Hünermann syndrome .
Catalyzed reaction
⇒ 
Zymosterol is rearranged to 5α-Cholesta-7,24-dien-3β-ol.
Individual evidence
Web links
Wikibooks: Biochemistry and Pathobiochemistry: Cholesterol Biosynthesis - Learning and Teaching Materials
- D'Eustachio / reactome: Zymosterol is isomerized to cholesta-7,24-dien-3beta-ol
- OrphaNet: X-linked dominant chondrodysplasia punctata