Gene/Proteome Database (LMPD)
LMPD ID
LMP001581
Gene ID
Species
Homo sapiens (Human)
Gene Name
cytochrome P450, family 11, subfamily B, polypeptide 2
Gene Symbol
Synonyms
ALDOS; CPN2; CYP11B; CYP11BL; CYPXIB2; P-450C18; P450C18; P450aldo
Alternate Names
cytochrome P450 11B2, mitochondrial; cytochrome P-450C18; aldosterone synthase; cytochrome P-450Aldo; steroid 11-beta-monooxygenase; steroid 11-beta/18-hydroxylase; aldosterone-synthesizing enzyme; steroid 18-hydroxylase, aldosterone synthase, P450C18, P450aldo; mitochondrial cytochrome P450, family 11, subfamily B, polypeptide 2; cytochrome P450, subfamily XIB (steroid 11-beta-hydroxylase), polypeptide 2
Chromosome
8
Map Location
8q21-q22
EC Number
1.14.15.4
Summary
This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This protein localizes to the mitochondrial inner membrane. The enzyme has steroid 18-hydroxylase activity to synthesize aldosterone and 18-oxocortisol as well as steroid 11 beta-hydroxylase activity. Mutations in this gene cause corticosterone methyl oxidase deficiency. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| cytochrome P450 11B2, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_000489 |
| Protein GI | 119829183 |
| UniProt ID | P19099 |
| mRNA ID | NM_000498 |
| Length | 503 |
| RefSeq Status | REVIEWED |
| MALRAKAEVCVAAPWLSLQRARALGTRAARAPRTVLPFEAMPQHPGNRWLRLLQIWREQGYEHLHLEMHQTFQELGPIFRYNLGGPRMVCVMLPEDVEKLQQVDSLHPCRMILEPWVAYRQHRGHKCGVFLLNGPEWRFNRLRLNPDVLSPKAVQRFLPMVDAVARDFSQALKKKVLQNARGSLTLDVQPSIFHYTIEASNLALFGERLGLVGHSPSSASLNFLHALEVMFKSTVQLMFMPRSLSRWISPKVWKEHFEAWDCIFQYGDNCIQKIYQELAFNRPQHYTGIVAELLLKAELSLEAIKANSMELTAGSVDTTAFPLLMTLFELARNPDVQQILRQESLAAAASISEHPQKATTELPLLRAALKETLRLYPVGLFLERVVSSDLVLQNYHIPAGTLVQVFLYSLGRNAALFPRPERYNPQRWLDIRGSGRNFHHVPFGFGMRQCLGRRLAEAEMLLLLHHVLKHFLVETLTQEDIKMVYSFILRPGTSPLLTFRAIN | |
| transit_peptide: 1..24 calculated_mol_wt: 2625 peptide sequence: MALRAKAEVCVAAPWLSLQRARAL mat_peptide: 25..503 product: cytochrome P450 11B2, mitochondrial calculated_mol_wt: 54953 peptide sequence: GTRAARAPRTVLPFEAMPQHPGNRWLRLLQIWREQGYEHLHLEMHQTFQELGPIFRYNLGGPRMVCVMLPEDVEKLQQVDSLHPCRMILEPWVAYRQHRGHKCGVFLLNGPEWRFNRLRLNPDVLSPKAVQRFLPMVDAVARDFSQALKKKVLQNARGSLTLDVQPSIFHYTIEASNLALFGERLGLVGHSPSSASLNFLHALEVMFKSTVQLMFMPRSLSRWISPKVWKEHFEAWDCIFQYGDNCIQKIYQELAFNRPQHYTGIVAELLLKAELSLEAIKANSMELTAGSVDTTAFPLLMTLFELARNPDVQQILRQESLAAAASISEHPQKATTELPLLRAALKETLRLYPVGLFLERVVSSDLVLQNYHIPAGTLVQVFLYSLGRNAALFPRPERYNPQRWLDIRGSGRNFHHVPFGFGMRQCLGRRLAEAEMLLLLHHVLKHFLVETLTQEDIKMVYSFILRPGTSPLLTFRAIN | |
Gene Information
Entrez Gene ID
Gene Name
cytochrome P450, family 11, subfamily B, polypeptide 2
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005743 | IC:BHF-UCL | C | mitochondrial inner membrane |
| GO:0005739 | IDA:BHF-UCL | C | mitochondrion |
| GO:0047783 | IEA:UniProtKB-EC | F | corticosterone 18-monooxygenase activity |
| GO:0020037 | IDA:UniProtKB | F | heme binding |
| GO:0005506 | IEA:InterPro | F | iron ion binding |
| GO:0004507 | IDA:UniProtKB | F | steroid 11-beta-monooxygenase activity |
| GO:0006700 | IDA:BHF-UCL | P | C21-steroid hormone biosynthetic process |
| GO:0032342 | IDA:UniProtKB | P | aldosterone biosynthetic process |
| GO:0032870 | IEP:UniProtKB | P | cellular response to hormone stimulus |
| GO:0035865 | IEP:UniProtKB | P | cellular response to potassium ion |
| GO:0034651 | IMP:UniProtKB | P | cortisol biosynthetic process |
| GO:0006705 | TAS:Reactome | P | mineralocorticoid biosynthetic process |
| GO:0055075 | IMP:BHF-UCL | P | potassium ion homeostasis |
| GO:0002017 | IMP:BHF-UCL | P | regulation of blood volume by renal aldosterone |
| GO:0003091 | IC:BHF-UCL | P | renal water homeostasis |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0055078 | IMP:BHF-UCL | P | sodium ion homeostasis |
| GO:0008202 | TAS:Reactome | P | steroid metabolic process |
| GO:0016125 | TAS:Reactome | P | sterol metabolic process |
| GO:0006805 | TAS:Reactome | P | xenobiotic metabolic process |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa00140 | Steroid hormone biosynthesis |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| PWY66-382 | mineralocorticoid biosynthesis |
Domain Information
UniProt Annotations
Entry Information
Gene Name
cytochrome P450, family 11, subfamily B, polypeptide 2
Protein Entry
C11B2_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | A steroid + 2 reduced adrenodoxin + O(2) + 2 H(+) = an 11-beta- hydroxysteroid + 2 oxidized adrenodoxin + H(2)O. |
| Catalytic Activity | Corticosterone + 2 reduced adrenodoxin + O(2) + 2 H(+) = 18-hydroxycorticosterone + 2 oxidized adrenodoxin + H(2)O. |
| Cofactor | Name=heme; Xref=ChEBI |
| Disease | Corticosterone methyloxidase 1 deficiency (CMO-1 deficiency) [MIM |
| Disease | Corticosterone methyloxidase 2 deficiency (CMO-2 deficiency) [MIM |
| Disease | Familial hyperaldosteronism 1 (FH1) [MIM |
| Function | Preferentially catalyzes the conversion of 11- deoxycorticosterone to aldosterone via corticosterone and 18- hydroxycorticosterone. |
| Similarity | Belongs to the cytochrome P450 family. |
| Subcellular Location | Mitochondrion membrane. |
| Web Resource | Name=Wikipedia; Note=CYP11B2 entry; URL="http://en.wikipedia.org/wiki/CYP11B2"; |
Identical and Related Proteins
Unique RefSeq proteins for LMP001581 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 119829183 | RefSeq | NP_000489 | 503 | cytochrome P450 11B2, mitochondrial precursor |
Identical Sequences to LMP001581 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:119829183 | DBBJ | BAG73068.1 | 503 | cytochrome P450, family 11, subfamily B, polypeptide 2, partial [synthetic construct] |
| GI:119829183 | GenBank | AAI11459.1 | 503 | CYP11B2 protein, partial [synthetic construct] |
| GI:119829183 | GenBank | EAW82292.1 | 503 | cytochrome P450, family 11, subfamily B, polypeptide 2 [Homo sapiens] |
| GI:119829183 | GenBank | ACA05912.1 | 503 | mitochondrial cytochrome P450, family 11, subfamily B, polypeptide 2 [Homo sapiens] |
| GI:119829183 | GenBank | AED82058.1 | 503 | Sequence 795 from patent US 7919467 |
| GI:119829183 | SwissProt | P19099.3 | 503 | RecName: Full=Cytochrome P450 11B2, mitochondrial; AltName: Full=Aldosterone synthase; Short=ALDOS; AltName: Full=Aldosterone-synthesizing enzyme; AltName: Full=CYPXIB2; AltName: Full=Cytochrome P-450Aldo; AltName: Full=Cytochrome P-450C18; AltName: Full=Steroid 18-hydroxylase; Flags: Precursor [Homo sapiens] |
Related Sequences to LMP001581 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:119829183 | EMBL | CAA38539.1 | 503 | aldosterone synthase (P-450aldo) [Homo sapiens] |
| GI:119829183 | GenBank | AAA35741.1 | 503 | 11-beta-hydroxylase-like-enzyme [Homo sapiens] |
| GI:119829183 | GenBank | AHD69434.1 | 503 | Sequence 448 from patent US 8586006 |
| GI:119829183 | RefSeq | XP_003282397.1 | 503 | PREDICTED: cytochrome P450 11B2, mitochondrial-like isoform 1 [Nomascus leucogenys] |
| GI:119829183 | RefSeq | XP_004047649.1 | 503 | PREDICTED: cytochrome P450 11B1, mitochondrial-like isoform 1 [Gorilla gorilla gorilla] |
| GI:119829183 | RefSeq | XP_010383781.1 | 503 | PREDICTED: cytochrome P450 11B1, mitochondrial-like isoform X1 [Rhinopithecus roxellana] |