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Protein Page:
CYP11B1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
CYP11B1 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. Defects in CYP11B1 are the cause of adrenal hyperplasia type 4 (AH4). AH4 is a form of congenital adrenal hyperplasia, a common recessive disease due to defective synthesis of cortisol. Congenital adrenal hyperplasia is characterized by androgen excess leading to ambiguous genitalia in affected females, rapid somatic growth during childhood in both sexes with premature closure of the epiphyses and short adult stature. Four clinical types: salt wasting (SW, the most severe type), simple virilizing (SV, less severely affected patients), with normal aldosterone biosynthesis, non-classic form or late onset (NC or LOAH), and cryptic (asymptomatic). AH4 patients usually have hypertension. Defects in CYP11B1 are a cause of familial hyperaldosteronism type 1 (FH1). It is a disorder characterized by hypertension, variable hyperaldosteronism, and abnormal adrenal steroid production, including 18-oxocortisol and 18-hydroxycortisol. There is significant phenotypic heterogeneity, and some individuals never develop hypertension. The molecular defect causing hyperaldosteronism familial type 1 is an anti-Lepore-type fusion of the CYP11B1 and CYP11B2 genes. The hybrid gene has the promoting part of CYP11B1, ACTH-sensitive, and the coding part of CYP11B2. Belongs to the cytochrome P450 family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 1.14.15.4; Oxidoreductase; Lipid Metabolism - androgen and estrogen; Lipid Metabolism - C21-steroid hormone; Mitochondrial
Cellular Component: mitochondrion; mitochondrial inner membrane
Molecular Function: steroid 11-beta-monooxygenase activity; iron ion binding; heme binding
Biological Process: steroid metabolic process; regulation of blood pressure; xenobiotic metabolic process; mineralocorticoid biosynthetic process; C21-steroid hormone biosynthetic process; immune response; glucose homeostasis; glucocorticoid biosynthetic process; sterol metabolic process; aldosterone biosynthetic process; cellular response to hormone stimulus
Reference #:  P15538 (UniProtKB)
Alt. Names/Synonyms: C11B1; CPN1; CYP11B; CYP11B1; CYPXIB1; Cytochrome P-450c11; Cytochrome P450 11B1, mitochondrial; cytochrome p450 XIB1; cytochrome P450, family 11, subfamily B, polypeptide 1; cytochrome P450, subfamily XIB (steroid 11-beta-hydroxylase), polypeptide 1; Cytochrome P450C11; DKFZp686B05283; FHI; FLJ36771; P450C11; S11BH; Steroid 11-beta-hydroxylase; steroid 11-beta-monooxygenase
Gene Symbols: CYP11B1
Molecular weight: 57,573 Da
Basal Isoelectric point: 9.4  Predict pI for various phosphorylation states
Select Structure to View Below

CYP11B1

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 Y81-p ELGPIFRyDLGGAGM
0 1 R120 LEPWVAYRQHRGHKC
0 1 R123 WVAYRQHRGHKCGVF
0 1 S150-p RLNPEVLsPNAVQRF
0 1 S169-p DAVARDFsQALKKKV
  mouse

 
H83 ELGPIFRHSVGKTQI
R122-m2 LESWIVHrELrGLGR
R125-m2 WIVHrELrGLGRGVF
S152 QLNPNVLSPKAVQKF
V171 DGIARDFVDNLKKKM
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