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cytochrome P450 11A1 (CYP11A1, cholesterol 20,22 desmolase, cytochrome P450 SCC, CYP11A1)
Function:
- catalyzes all three steps in cleavage of cholesterol to pregnenolone & isocaproic acid
- lipid metabolism; C21-steroid hormone metabolism
Structure: belongs to the cytochrome P450 family
Compartment: mitochondria
Expression: induced by 8-bromo cyclic AMP
Pathology:
- defects in CYP11A1 are a cause of
a) congenital adrenal insufficiency
b) congenital lipoid adrenal hyperplasia (CLAH)
- inhibition of CYP11A1 may improve survival in castration-resistant prostate cancer (investigational) [3]
Related
adrenal hyperplasia 1; congenital lipoid adrenal hyperplasia (CLAH)
cholesterol desmolase gene or CYP11A
steroid biosynthesis
General
cytochrome P450
mitochondrial membrane protein
Properties
SIZE: MW = 60 kD
entity length = 521 aa
COMPARTMENT: inner mitochondrial membrane
MOTIF: Iron [Fe]-binding site
SITE: 462-462
Database Correlations
OMIM correlations
MORBIDMAP 118485
UniProt P05108
Pfam PF00067
Entrez Gene 1583
KEGG correlations
ENZYME 1.14.15.6
References
- OMIM [201710 118485]
- UniProt :accession P05108
- Fizazi K et al.
Targeted inhibition of CYP11A1 in castration-resistant prostate cancer.
NEJM Evid 2024 Jan; 3:EVIDoa2300171.
PMID: 38320513 PMCID: PMC10852404 Free PMC article.
https://evidence.nejm.org/doi/10.1056/EVIDoa2300171
Component-of
multienzyme complex