General Information of Drug-Metabolizing Enzyme (DME) (ID: DE0JMZ5)

DME Name Steroid 21-hydroxylase (CYP21A2)
Synonyms Cytochrome P-450c21; Cytochrome P450 21; Cytochrome P450 XXI; Cytochrome P450-C21; Cytochrome P450-C21B; 21-OHase; CYP21; CYP21B
Gene Name CYP21A2
UniProt ID
CP21A_HUMAN
INTEDE ID
DME0037
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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Gene ID
1589
EC Number EC: 1.14.14.16
Oxidoreductase
Oxygen paired donor oxidoreductase
Flavin/flavoprotein donor oxidoreductase
EC: 1.14.14.16
Lineage Species: Homo sapiens
Kingdom: Metazoa
Phylum: Chordata
Class: Mammalia
Order: Primates
Family: Hominidae
Genus: Homo
Species: Homo sapiens
Sequence
MLLLGLLLLPLLAGARLLWNWWKLRSLHLPPLAPGFLHLLQPDLPIYLLGLTQKFGPIYR
LHLGLQDVVVLNSKRTIEEAMVKKWADFAGRPEPLTYKLVSKNYPDLSLGDYSLLWKAHK
KLTRSALLLGIRDSMEPVVEQLTQEFCERMRAQPGTPVAIEEEFSLLTCSIICYLTFGDK
IKDDNLMPAYYKCIQEVLKTWSHWSIQIVDVIPFLRFFPNPGLRRLKQAIEKRDHIVEMQ
LRQHKESLVAGQWRDMMDYMLQGVAQPSMEEGSGQLLEGHVHMAAVDLLIGGTETTANTL
SWAVVFLLHHPEIQQRLQEELDHELGPGASSSRVPYKDRARLPLLNATIAEVLRLRPVVP
LALPHRTTRPSSISGYDIPEGTVIIPNLQGAHLDETVWERPHEFWPDRFLEPGKNSRALA
FGCGARVCLGEPLARLELFVVLTRLLQAFTLLPSGDALPSLQPLPHCSVILKMQPFQVRL
QPRGMGAHSPGQNQ
Function This enzyme specifically catalyzes the 21-hydroxylation of steroids.
KEGG Pathway
Aldosterone synthesis and secretion (hsa04925 )
Cortisol synthesis and secretion (hsa04927 )
Cushing syndrome (hsa04934 )
Metabolic pathways (hsa01100 )
Steroid hormone biosynthesis (hsa00140 )
Reactome Pathway
Endogenous sterols (R-HSA-211976 )
Glucocorticoid biosynthesis (R-HSA-194002 )
Mineralocorticoid biosynthesis (R-HSA-193993 )
Defective CYP21A2 causes Adrenal hyperplasia 3 (AH3) (R-HSA-5579021 )

Molecular Interaction Atlas (MIA) of This DME

Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DME
2 Approved Drug(s) Metabolized by This DME
Drug Name Drug ID Indication ICD 11 Highest Status REF
Hydroxyprogesterone DMIKQH5 Solid tumour/cancer 2A00-2F9Z Approved [1]
Progesterone DMUY35B Amenorrhea GA20.0 Approved [2]

References

1 Functional characterization of two novel point mutations in the CYP21 gene causing simple virilizing forms of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2005 Jan;90(1):445-54.
2 Human cytochrome P450 21A2, the major steroid 21-hydroxylase: structure of the enzyme progesterone substrate complex and rate-limiting C-H bond cleavage. J Biol Chem. 2015 May 22;290(21):13128-43.