Gene/Proteome Database (LMPD)

LMPD ID
LMP002086
Gene ID
1584
Species
Homo sapiens (Human)
Gene Name
cytochrome P450, family 11, subfamily B, polypeptide 1
Gene Symbol
CYP11B1
Synonyms
CPN1; CYP11B; FHI; P450C11
Alternate Names
cytochrome P450 11B1, mitochondrial; CYPXIB1; cytochrome P450C11; cytochrome P-450c11; cytochrome p450 XIB1; steroid 11-beta-hydroxylase; steroid 11-beta-monooxygenase; cytochrome P450, subfamily XIB (steroid 11-beta-hydroxylase), polypeptide 1
Chromosome
8
Map Location
8q21
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 and is involved in the conversion of progesterone to cortisol in the adrenal cortex. Mutations in this gene cause congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency. Transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for CYP11B1

Proteins

cytochrome P450 11B1, mitochondrial isoform 1 precursor
Refseq ID NP_000488
Protein GI 61743918
UniProt ID P15538
mRNA ID NM_000497
Length 503
RefSeq Status REVIEWED
MALRAKAEVCMAVPWLSLQRAQALGTRAARVPRTVLPFEAMPRRPGNRWLRLLQIWREQGYEDLHLEVHQTFQELGPIFRYDLGGAGMVCVMLPEDVEKLQQVDSLHPHRMSLEPWVAYRQHRGHKCGVFLLNGPEWRFNRLRLNPEVLSPNAVQRFLPMVDAVARDFSQALKKKVLQNARGSLTLDVQPSIFHYTIEASNLALFGERLGLVGHSPSSASLNFLHALEVMFKSTVQLMFMPRSLSRWTSPKVWKEHFEAWDCIFQYGDNCIQKIYQELAFSRPQQYTSIVAELLLNAELSPDAIKANSMELTAGSVDTTVFPLLMTLFELARNPNVQQALRQESLAAAASISEHPQKATTELPLLRAALKETLRLYPVGLFLERVASSDLVLQNYHIPAGTLVRVFLYSLGRNPALFPRPERYNPQRWLDIRGSGRNFYHVPFGFGMRQCLGRRLAEAEMLLLLHHVLKHLQVETLTQEDIKMVYSFILRPSMFPLLTFRAIN
cytochrome P450 11B1, mitochondrial isoform 2 precursor
Refseq ID NP_001021384
Protein GI 71067343
UniProt ID P15538
mRNA ID NM_001026213
Length 437
RefSeq Status REVIEWED
MALRAKAEVCMAVPWLSLQRAQALGTRAARVPRTVLPFEAMPRRPGNRWLRLLQIWREQGYEDLHLEVHQTFQELGPIFRYDLGGAGMVCVMLPEDVEKLQQVDSLHPHRMSLEPWVAYRQHRGHKCGVFLLNGPEWRFNRLRLNPEVLSPNAVQRFLPMVDAVARDFSQALKKKVLQNARGSLTLDVQPSIFHYTIEASNLALFGERLGLVGHSPSSASLNFLHALEVMFKSTVQLMFMPRSLSRWTSPKVWKEHFEAWDCIFQYGDNCIQKIYQELAFSRPQQYTSIVAELLLNAELSPDAIKANSMELTAGSVDTTVFPLLMTLFELARNPNVQQALRQESLAAAASISEHPQKATTELPLLRAALKETLRLYPVGLFLERVASSDLVLQNYHIPAGVLKHLQVETLTQEDIKMVYSFILRPSMFPLLTFRAIN
transit_peptide: 1..24 calculated_mol_wt: 2657 peptide sequence: MALRAKAEVCMAVPWLSLQRAQAL mat_peptide: 25..503 product: cytochrome P450 11B1, mitochondrial isoform 1 calculated_mol_wt: 54934 peptide sequence: GTRAARVPRTVLPFEAMPRRPGNRWLRLLQIWREQGYEDLHLEVHQTFQELGPIFRYDLGGAGMVCVMLPEDVEKLQQVDSLHPHRMSLEPWVAYRQHRGHKCGVFLLNGPEWRFNRLRLNPEVLSPNAVQRFLPMVDAVARDFSQALKKKVLQNARGSLTLDVQPSIFHYTIEASNLALFGERLGLVGHSPSSASLNFLHALEVMFKSTVQLMFMPRSLSRWTSPKVWKEHFEAWDCIFQYGDNCIQKIYQELAFSRPQQYTSIVAELLLNAELSPDAIKANSMELTAGSVDTTVFPLLMTLFELARNPNVQQALRQESLAAAASISEHPQKATTELPLLRAALKETLRLYPVGLFLERVASSDLVLQNYHIPAGTLVRVFLYSLGRNPALFPRPERYNPQRWLDIRGSGRNFYHVPFGFGMRQCLGRRLAEAEMLLLLHHVLKHLQVETLTQEDIKMVYSFILRPSMFPLLTFRAIN transit_peptide: 1..24 calculated_mol_wt: 2657 peptide sequence: MALRAKAEVCMAVPWLSLQRAQAL mat_peptide: 25..437 product: cytochrome P450 11B1, mitochondrial isoform 2 calculated_mol_wt: 47111 peptide sequence: GTRAARVPRTVLPFEAMPRRPGNRWLRLLQIWREQGYEDLHLEVHQTFQELGPIFRYDLGGAGMVCVMLPEDVEKLQQVDSLHPHRMSLEPWVAYRQHRGHKCGVFLLNGPEWRFNRLRLNPEVLSPNAVQRFLPMVDAVARDFSQALKKKVLQNARGSLTLDVQPSIFHYTIEASNLALFGERLGLVGHSPSSASLNFLHALEVMFKSTVQLMFMPRSLSRWTSPKVWKEHFEAWDCIFQYGDNCIQKIYQELAFSRPQQYTSIVAELLLNAELSPDAIKANSMELTAGSVDTTVFPLLMTLFELARNPNVQQALRQESLAAAASISEHPQKATTELPLLRAALKETLRLYPVGLFLERVASSDLVLQNYHIPAGVLKHLQVETLTQEDIKMVYSFILRPSMFPLLTFRAIN

Gene Information

Entrez Gene ID
1584
Gene Name
cytochrome P450, family 11, subfamily B, polypeptide 1
Gene Symbol
CYP11B1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005743 TAS:BHF-UCL C mitochondrial inner membrane
GO:0005739 IDA:BHF-UCL C mitochondrion
GO:0020037 IC:BHF-UCL F heme binding
GO:0005506 IEA:InterPro F iron ion binding
GO:0004507 IDA:BHF-UCL F steroid 11-beta-monooxygenase activity
GO:0006700 IDA:BHF-UCL P C21-steroid hormone biosynthetic process
GO:0032342 IMP: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:0006704 TAS:Reactome P glucocorticoid biosynthetic process
GO:0042593 TAS:BHF-UCL P glucose homeostasis
GO:0006955 TAS:BHF-UCL P immune response
GO:0006705 TAS:Reactome P mineralocorticoid biosynthetic process
GO:0008217 IMP:BHF-UCL P regulation of blood pressure
GO:0044281 TAS:Reactome P small molecule metabolic process
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

InterPro Annotations

Accession Description
IPR001128 Cytochrome P450
IPR017972 Cytochrome P450, conserved site
IPR002399 Cytochrome P450, mitochondrial

UniProt Annotations

Entry Information

Gene Name
cytochrome P450, family 11, subfamily B, polypeptide 1
Protein Entry
C11B1_HUMAN
UniProt ID
P15538
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P15538-1; Sequence=Displayed; Name=2; IsoId=P15538-2; Sequence=VSP_043308; Note=No experimental confirmation available.;
Catalytic Activity A steroid + 2 reduced adrenodoxin + O(2) + 2 H(+) = an 11-beta- hydroxysteroid + 2 oxidized adrenodoxin + H(2)O.
Cofactor Name=heme; Xref=ChEBI
Disease Adrenal hyperplasia 4 (AH4) [MIM
Disease Familial hyperaldosteronism 1 (FH1) [MIM
Function Has steroid 11-beta-hydroxylase activity. In addition to this activity, the 18 or 19-hydroxylation of steroids and the aromatization of androstendione to estrone have also been ascribed to cytochrome P450 XIB.
Similarity Belongs to the cytochrome P450 family.
Subcellular Location Mitochondrion membrane.

Identical and Related Proteins

Unique RefSeq proteins for LMP002086 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
61743918 RefSeq NP_000488 503 cytochrome P450 11B1, mitochondrial isoform 1 precursor
71067343 RefSeq NP_001021384 437 cytochrome P450 11B1, mitochondrial isoform 2 precursor

Identical Sequences to LMP002086 proteins

Reference Database Accession Length Protein Name
GI:61743918 GenBank AAH96287.1 503 Cytochrome P450, family 11, subfamily B, polypeptide 1 [Homo sapiens]
GI:71067343 GenBank AAH96286.1 437 Cytochrome P450, family 11, subfamily B, polypeptide 1 [Homo sapiens]
GI:61743918 GenBank EAW82293.1 503 cytochrome P450, family 11, subfamily B, polypeptide 1, isoform CRA_a [Homo sapiens]
GI:61743918 GenBank ABY87528.1 503 cytochrome P450, family 11, subfamily B, polypeptide 1 [Homo sapiens]
GI:61743918 GenBank AHD69432.1 503 Sequence 446 from patent US 8586006
GI:71067343 GenBank AHD69433.1 437 Sequence 447 from patent US 8586006
GI:61743918 SwissProt P15538.5 503 RecName: Full=Cytochrome P450 11B1, mitochondrial; AltName: Full=CYPXIB1; AltName: Full=Cytochrome P-450c11; Short=Cytochrome P450C11; AltName: Full=Steroid 11-beta-hydroxylase; Flags: Precursor [Homo sapiens]

Related Sequences to LMP002086 proteins

Reference Database Accession Length Protein Name
GI:61743918 EMBL CAA39290.1 503 unnamed protein product [Homo sapiens]
GI:61743918 GenBank AAA52149.1 503 steroid 11-beta-hydroxylase [Homo sapiens]
GI:61743918 GenBank AAL84813.1 503 mutated cytochrome P-450c11 protein [Homo sapiens]
GI:61743918 GenBank EAW82294.1 526 cytochrome P450, family 11, subfamily B, polypeptide 1, isoform CRA_b [Homo sapiens]
GI:61743918 GenBank EAW82295.1 548 cytochrome P450, family 11, subfamily B, polypeptide 1, isoform CRA_c [Homo sapiens]
GI:71067343 GenBank AHD69432.1 503 Sequence 446 from patent US 8586006
GI:71067343 RefSeq NP_000488.3 503 cytochrome P450 11B1, mitochondrial isoform 1 precursor [Homo sapiens]
GI:71067343 RefSeq XP_001084088.1 437 PREDICTED: cytochrome P450 11B1, mitochondrial-like isoform 1 [Macaca mulatta]
GI:61743918 RefSeq XP_002819552.1 503 PREDICTED: cytochrome P450 11B1, mitochondrial isoform X1 [Pongo abelii]
GI:71067343 RefSeq XP_002819554.1 437 PREDICTED: cytochrome P450 11B1, mitochondrial isoform X2 [Pongo abelii]
GI:71067343 RefSeq XP_005564255.1 437 PREDICTED: cytochrome P450 11B1, mitochondrial-like isoform X2 [Macaca fascicularis]
GI:71067343 SwissProt P15538.5 503 RecName: Full=Cytochrome P450 11B1, mitochondrial; AltName: Full=CYPXIB1; AltName: Full=Cytochrome P-450c11; Short=Cytochrome P450C11; AltName: Full=Steroid 11-beta-hydroxylase; Flags: Precursor [Homo sapiens]