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beta,beta-carotene 15,15'-monooxygenase; beta-carotene dioxygenase 1 (BCMO1, BCDO, BCDO1)
Function:
1) vitamin A biosynthesis
2) symmetrically cleaves beta-carotene into retinal (2)
3) 3 steps
a) epoxidation of the 15,15'-double bond
b) hydration of the double bond leading to ring opening
c) oxidative cleavage of the diol formed
beta-carotene + O2 2 retinal
Cofactor: binds 1 Fe+2 ion per subunit (putative)
Structure: belongs to the carotenoid oxygenase family
Expression:
- expressed in retinal pigment epithelium > kidney, testis, liver, brain, small intestine, colon
Pathology:
- defects in BCMO1 are the cause of autosomal dominant hypercarotenemia & vitamin A deficiency
General
hydroxylase; monooxygenase
Properties
SIZE: entity length = 547 aa
MW = 63 kD
MOTIF: Iron [Fe]-binding site
SITE: 172-172
Iron [Fe]-binding site
SITE: 237-237
active site
Iron [Fe]-binding site
SITE: 308-308
Iron [Fe]-binding site
SITE: 514-514
Database Correlations
OMIM correlations
MORBIDMAP 605748
UniProt Q9HAY6
Pfam PF03055
Entrez Gene 53630
Kegg hsa:53630
ENZYME 1.14.99.36
References
UniProt :accession Q9HAY6