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Protein Page:
CYP11A1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
CYP11A1 Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone. Defects in CYP11A1 are the cause of adrenal insufficiency congenital with 46,XY sex reversal (AICSR). A rare disorder that can present as acute adrenal insufficiency in infancy or childhood. ACTH and plasma renin activity are elevated and adrenal steroids are inappropriately low or absent; the 46,XY patients have female external genitalia, sometimes with clitoromegaly. The phenotypic spectrum ranges from prematurity, complete underandrogenization, and severe early-onset adrenal failure to term birth with clitoromegaly and later-onset adrenal failure. Patients with congenital adrenal insufficiency do not manifest the massive adrenal enlargement typical of congenital lipoid adrenal hyperplasia. Belongs to the cytochrome P450 family. Note: This description may include information from UniProtKB.
Protein type: Lipid Metabolism - C21-steroid hormone; EC 1.14.15.6; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 15q23-q24
Cellular Component: mitochondrial crista; mitochondrion; mitochondrial matrix; perikaryon
Molecular Function: cholesterol monooxygenase (side-chain-cleaving) activity; iron ion binding; heme binding; cholesterol binding
Biological Process: steroid metabolic process; response to alkaloid; maternal process involved in pregnancy; dibenzo-p-dioxin metabolic process; response to insecticide; estrogen biosynthetic process; response to L-ascorbic acid; response to salt stress; response to vitamin E; Leydig cell differentiation; biphenyl metabolic process; response to gamma radiation; cerebellum development; response to corticosterone stimulus; response to drug; cholesterol metabolic process; progesterone biosynthetic process; fractalkine metabolic process; phthalate metabolic process; hippocampus development; mating behavior; vitamin D metabolic process; Schwann cell differentiation; response to genistein; response to hydrogen peroxide; xenobiotic metabolic process; C21-steroid hormone biosynthetic process; sterol metabolic process; granulosa cell differentiation; phenol metabolic process
Disease: Adrenal Insufficiency, Congenital, With 46,xy Sex Reversal, Partial Or Complete
Reference #:  P05108 (UniProtKB)
Alt. Names/Synonyms: cholesterol 20-22 desmolase; Cholesterol desmolase; cholesterol monooxygenase (side-chain cleaving); Cholesterol side-chain cleavage enzyme, mitochondrial; CP11A; CYP11A; CYP11A1; CYPXIA1; Cytochrome P450 11A1; Cytochrome P450(scc); cytochrome P450, family 11, subfamily A, polypeptide 1; cytochrome P450, subfamily XIA (cholesterol side chain cleavage); cytochrome P450C11A1; P450SCC; steroid 20-22-lyase
Gene Symbols: CYP11A1
Molecular weight: 60,102 Da
Basal Isoelectric point: 8.89  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
Select Structure to View Below

CYP11A1

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


 LTP 

LTP: 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.


 HTP 

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


       human

 
0 1 S113-p DVALLFKsEGPNPER
0 1 T164-p EVMAPEAtKNFLPLL
0 1 N193 IKKAGSGNYSGDISD
0 1 T369 QAQGDMATMLQLVPL
0 1 K382 PLLKASIKETLRLHP
0 1 N434 TFFFDPENFDPTRWL
  mouse

 
C110 DASILFSCEGPNPER
I161 EVMAPGAIKNFVPLL
N190 IKQQNSGNFSGVISD
K365 QAQGDMAKMVQLVPL
K378 PLLKASIKETLRLHP
K430 GFFPNPNKFDPTRWL
  rat

 
C111 DAATLFSCEGPNPER
I162 EVMAPDSIKNFVPLL
K191-ac IKQQNSGkFSGDISD
K366-ac QAQGDMAkMVQLVPL
K379-ac PLLKASIkETLRLHP
K431-ac SFFPNPNkFDPTRWL
  cow

 
F113 DVAHLFKFEGSYPER
I164 EVMAPEAIKNFIPLL
- under review  
K368 QAEGDISKMLQMVPL
K381 PLLKASIKETLRLHP
K433 AFFSSPDKFDPTRWL
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