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Protein Page:
OTC (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
OTC Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD). OTCD is an X- linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms. Belongs to the ATCase/OTCase family. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial; Transferase; Amino Acid Metabolism - arginine and proline; EC 2.1.3.3
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane
Molecular Function: amino acid binding; ornithine carbamoyltransferase activity; phospholipid binding; phosphate binding
Biological Process: response to drug; citrulline biosynthetic process; arginine biosynthetic process; response to zinc ion; midgut development; liver development; ornithine catabolic process; urea cycle; response to insulin stimulus
Reference #:  P00480 (UniProtKB)
Alt. Names/Synonyms: MGC129967; MGC129968; MGC138856; OCTD; ornithine carbamoyltransferase; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; OTC; OTCase
Gene Symbols: OTC
Molecular weight: 39,935 Da
Basal Isoelectric point: 8.75  Predict pI for various phosphorylation states
Select Structure to View Below

OTC

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 K38 LQNKVQLKGRDLLTL
0 1 K38 LQNKVQLKGRDLLTL
1 1 K46-ac GRDLLTLkNFTGEEI
0 1 Y55-p FTGEEIKyMLWLSAD
0 8 K70 LKFRIKQKGEYLPLL
0 2 K70 LKFRIKQKGEYLPLL
0 1 K80 YLPLLQGKSLGMIFE
1 35 K88-ac SLGMIFEkRSTRTRL
0 2 K88 SLGMIFEKRSTRTRL
0 1 S133-p DTARVLSsMADAVLA
0 5 K144 AVLARVYKQsDLDTL
0 1 K144 AVLARVYKQsDLDTL
0 2 K144 AVLARVYKQsDLDTL
0 1 S146-p LARVYKQsDLDTLAK
0 18 K221 HLQAATPKGyEPDAS
0 1 K221 HLQAATPKGyEPDAS
0 2 K221 HLQAATPKGyEPDAS
0 1 Y223-p QAATPKGyEPDASVT
1 3 K231-ac EPDASVTkLAEQYAK
0 1 K231 EPDASVTKLAEQYAK
0 2 K231 EPDASVTKLAEQYAK
0 19 K238 kLAEQYAKENGTKLL
0 1 K238 kLAEQYAKENGTKLL
0 2 K238 kLAEQYAKENGTKLL
0 2 K243 YAKENGTKLLLTNDP
0 1 K243 YAKENGTKLLLTNDP
0 1 K243 YAKENGTKLLLTNDP
0 8 K274 SMGQEEEKKKRLQAF
0 1 K274 SMGQEEEKKKRLQAF
0 9 K275 MGQEEEKKKRLQAFQ
0 2 K276 GQEEEKKKRLQAFQG
0 1 Y284-p RLQAFQGyQVTMKTA
0 1 K289 QGyQVTMKTAKVAAS
0 1 K289 QGyQVTMKTAKVAAS
0 1 K292 QVTMKTAKVAASDWT
0 1 K292 QVTMKTAKVAASDWT
0 1 K292 QVTMKTAKVAASDWT
0 2 K307 FLHCLPRKPEEVDDE
0 1 K307 FLHCLPRKPEEVDDE
0 1 Y317-p EVDDEVFySPRSLVF
0 1 Y345 MVSLLTDYSPQLQKP
  mouse

 
K38-ac VQSQVQLkGRDLLTL
K38-sc VQSQVQLkGRDLLTL
K46 GRDLLTLKNFTGEEI
Y55 FTGEEIQYMLWLSAD
K70-ac LKFRIKQkGEYLPLL
K70-sc LKFRIKQkGEYLPLL
K80-ac YLPLLQGkSLGMIFE
K88-ac SLGMIFEkRSTRTRL
K88-sc SLGMIFEkRSTRTRL
S133 DTARVLSSMTDAVLA
K144-ac AVLARVYkQSDLDTL
K144-ub AVLARVYkQSDLDTL
K144-sc AVLARVYkQSDLDTL
S146 LARVYkQSDLDTLAK
K221-ac HLQAATPkGYEPDPN
K221-ub HLQAATPkGYEPDPN
K221-sc HLQAATPkGYEPDPN
Y223 QAATPkGYEPDPNIV
K231-ac EPDPNIVkLAEQYAk
K231-ub EPDPNIVkLAEQYAk
K231-sc EPDPNIVkLAEQYAk
K238-ac kLAEQYAkENGTkLS
K238-ub kLAEQYAkENGTkLS
K238-sc kLAEQYAkENGTkLS
K243-ac YAkENGTkLSMTNDP
K243-ub YAkENGTkLSMTNDP
K243-sc YAkENGTkLSMTNDP
K274-ac SMGQEDEkkkRLQAF
K274-sc SMGQEDEkkkRLQAF
K275-ac MGQEDEkkkRLQAFQ
K276-ac GQEDEkkkRLQAFQG
Y284 RLQAFQGYQVTMkTA
K289-ub QGYQVTMkTAkVAAS
K289-sc QGYQVTMkTAkVAAS
K292-ac QVTMkTAkVAASDWT
K292-ub QVTMkTAkVAASDWT
K292-sc QVTMkTAkVAASDWT
K307-ac FLHCLPRkPEEVDDE
K307-sc FLHCLPRkPEEVDDE
Y317 EVDDEVFYSPRSLVF
Y345-p MVSLLTDySPVLQKP
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