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Protein

Ornithine carbamoyltransferase, mitochondrial

Gene

Otc

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalytic activityi

Carbamoyl phosphate + L-ornithine = phosphate + L-citrulline.

Enzyme regulationi

Negatively regulated by lysine acetylation.By similarity

Pathwayi: urea cycle

This protein is involved in step 1 of the subpathway that synthesizes L-citrulline from L-ornithine and carbamoyl phosphate.
Proteins known to be involved in this subpathway in this organism are:
  1. Ornithine carbamoyltransferase, mitochondrial (Otc)
This subpathway is part of the pathway urea cycle, which is itself part of Nitrogen metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes L-citrulline from L-ornithine and carbamoyl phosphate, the pathway urea cycle and in Nitrogen metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei141Carbamoyl phosphateBy similarity1
Binding sitei141OrnithineBy similarity1
Binding sitei168Carbamoyl phosphateBy similarity1
Binding sitei199OrnithineBy similarity1
Active sitei303By similarity1
Binding sitei330Carbamoyl phosphateBy similarity1
Binding sitei330OrnithineBy similarity1

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Amino-acid biosynthesis, Arginine biosynthesis, Urea cycle

Enzyme and pathway databases

ReactomeiR-MMU-70635. Urea cycle.
UniPathwayiUPA00158; UER00271.

Names & Taxonomyi

Protein namesi
Recommended name:
Ornithine carbamoyltransferase, mitochondrial (EC:2.1.3.3)
Alternative name(s):
Ornithine transcarbamylase
Short name:
OTCase
Gene namesi
Name:Otc
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome X

Organism-specific databases

MGIiMGI:97448. Otc.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: MGI
  • mitochondrial matrix Source: UniProtKB-SubCell
  • mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Defects in Otc are the cause of the Sparse fur (spf) phenotype. Spf mouse have an OTCase with an overall decrease in activity, and altered substrate affinity.

Keywords - Diseasei

Disease mutation

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 32MitochondrionAdd BLAST32
ChainiPRO_000002033533 – 354Ornithine carbamoyltransferase, mitochondrialAdd BLAST322

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei70N6-acetyllysine; alternateCombined sources1
Modified residuei70N6-succinyllysine; alternateCombined sources1
Modified residuei80N6-succinyllysineCombined sources1
Modified residuei88N6-acetyllysine; alternateCombined sources1
Modified residuei88N6-succinyllysine; alternateCombined sources1
Modified residuei133PhosphoserineBy similarity1
Modified residuei144N6-acetyllysine; alternateCombined sources1
Modified residuei144N6-succinyllysine; alternateCombined sources1
Modified residuei221N6-acetyllysine; alternateCombined sources1
Modified residuei221N6-succinyllysine; alternateCombined sources1
Modified residuei231N6-acetyllysine; alternateCombined sources1
Modified residuei231N6-succinyllysine; alternateCombined sources1
Modified residuei238N6-acetyllysine; alternateCombined sources1
Modified residuei238N6-succinyllysine; alternateCombined sources1
Modified residuei243N6-acetyllysineCombined sources1
Modified residuei274N6-succinyllysineCombined sources1
Modified residuei289N6-succinyllysineCombined sources1
Modified residuei292N6-acetyllysine; alternateCombined sources1
Modified residuei292N6-succinyllysine; alternateCombined sources1
Modified residuei307N6-acetyllysine; alternateCombined sources1
Modified residuei307N6-succinyllysine; alternateCombined sources1

Post-translational modificationi

Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

PaxDbiP11725.
PeptideAtlasiP11725.
PRIDEiP11725.

2D gel databases

SWISS-2DPAGEP11725.

PTM databases

iPTMnetiP11725.
PhosphoSitePlusiP11725.
SwissPalmiP11725.

Expressioni

Gene expression databases

BgeeiENSMUSG00000031173.
CleanExiMM_OTC.
ExpressionAtlasiP11725. baseline and differential.
GenevisibleiP11725. MM.

Interactioni

Subunit structurei

Homotrimer.

Protein-protein interaction databases

IntActiP11725. 2 interactors.
MINTiMINT-1838977.
STRINGi10090.ENSMUSP00000056152.

Structurei

3D structure databases

ProteinModelPortaliP11725.
SMRiP11725.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni90 – 94Carbamoyl phosphate bindingBy similarity5
Regioni263 – 267Ornithine bindingBy similarity5
Regioni302 – 305Ornithine bindingBy similarity4

Sequence similaritiesi

Belongs to the ATCase/OTCase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1504. Eukaryota.
COG0078. LUCA.
GeneTreeiENSGT00510000047417.
HOGENOMiHOG000022686.
HOVERGENiHBG007881.
InParanoidiP11725.
KOiK00611.
OMAiEHPCQIL.
OrthoDBiEOG091G0QB1.
PhylomeDBiP11725.
TreeFamiTF352580.

Family and domain databases

Gene3Di3.40.50.1370. 2 hits.
InterProiIPR006132. Asp/Orn_carbamoyltranf_P-bd.
IPR006130. Asp/Orn_carbamoylTrfase.
IPR006131. Asp_carbamoyltransf_Asp/Orn-bd.
IPR002292. Orn/put_carbamltrans.
[Graphical view]
PfamiPF00185. OTCace. 1 hit.
PF02729. OTCace_N. 1 hit.
[Graphical view]
PRINTSiPR00100. AOTCASE.
PR00102. OTCASE.
SUPFAMiSSF53671. SSF53671. 1 hit.
TIGRFAMsiTIGR00658. orni_carb_tr. 1 hit.
PROSITEiPS00097. CARBAMOYLTRANSFERASE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P11725-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLSNLRILLN NAALRKGHTS VVRHFWCGKP VQSQVQLKGR DLLTLKNFTG
60 70 80 90 100
EEIQYMLWLS ADLKFRIKQK GEYLPLLQGK SLGMIFEKRS TRTRLSTETG
110 120 130 140 150
FALLGGHPSF LTTQDIHLGV NESLTDTARV LSSMTDAVLA RVYKQSDLDT
160 170 180 190 200
LAKEASIPIV NGLSDLYHPI QILADYLTLQ EHYGSLKGLT LSWIGDGNNI
210 220 230 240 250
LHSIMMSAAK FGMHLQAATP KGYEPDPNIV KLAEQYAKEN GTKLSMTNDP
260 270 280 290 300
LEAARGGNVL ITDTWISMGQ EDEKKKRLQA FQGYQVTMKT AKVAASDWTF
310 320 330 340 350
LHCLPRKPEE VDDEVFYSPR SLVFPEAENR KWTIMAVMVS LLTDYSPVLQ

KPKF
Length:354
Mass (Da):39,765
Last modified:October 1, 1989 - v1
Checksum:i33BBE5D1E88AA196
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti195G → R in CAA30121 (PubMed:2831503).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural varianti117H → N in spf. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M17030 mRNA. Translation: AAA39865.1.
M12716 Genomic DNA. Translation: AAA39864.1. Sequence problems.
X07092
, X07093, X07094, X07095, X07096, X07097, X07098, X07099, X07100 Genomic DNA. Translation: CAA30121.1.
CCDSiCCDS30015.1.
PIRiA43609. OWMS.
RefSeqiNP_032795.1. NM_008769.4.
UniGeneiMm.2611.

Genome annotation databases

EnsembliENSMUST00000049910; ENSMUSP00000056152; ENSMUSG00000031173.
GeneIDi18416.
KEGGimmu:18416.
UCSCiuc009sqk.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M17030 mRNA. Translation: AAA39865.1.
M12716 Genomic DNA. Translation: AAA39864.1. Sequence problems.
X07092
, X07093, X07094, X07095, X07096, X07097, X07098, X07099, X07100 Genomic DNA. Translation: CAA30121.1.
CCDSiCCDS30015.1.
PIRiA43609. OWMS.
RefSeqiNP_032795.1. NM_008769.4.
UniGeneiMm.2611.

3D structure databases

ProteinModelPortaliP11725.
SMRiP11725.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP11725. 2 interactors.
MINTiMINT-1838977.
STRINGi10090.ENSMUSP00000056152.

PTM databases

iPTMnetiP11725.
PhosphoSitePlusiP11725.
SwissPalmiP11725.

2D gel databases

SWISS-2DPAGEP11725.

Proteomic databases

PaxDbiP11725.
PeptideAtlasiP11725.
PRIDEiP11725.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000049910; ENSMUSP00000056152; ENSMUSG00000031173.
GeneIDi18416.
KEGGimmu:18416.
UCSCiuc009sqk.1. mouse.

Organism-specific databases

CTDi5009.
MGIiMGI:97448. Otc.

Phylogenomic databases

eggNOGiKOG1504. Eukaryota.
COG0078. LUCA.
GeneTreeiENSGT00510000047417.
HOGENOMiHOG000022686.
HOVERGENiHBG007881.
InParanoidiP11725.
KOiK00611.
OMAiEHPCQIL.
OrthoDBiEOG091G0QB1.
PhylomeDBiP11725.
TreeFamiTF352580.

Enzyme and pathway databases

UniPathwayiUPA00158; UER00271.
ReactomeiR-MMU-70635. Urea cycle.

Miscellaneous databases

PROiP11725.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000031173.
CleanExiMM_OTC.
ExpressionAtlasiP11725. baseline and differential.
GenevisibleiP11725. MM.

Family and domain databases

Gene3Di3.40.50.1370. 2 hits.
InterProiIPR006132. Asp/Orn_carbamoyltranf_P-bd.
IPR006130. Asp/Orn_carbamoylTrfase.
IPR006131. Asp_carbamoyltransf_Asp/Orn-bd.
IPR002292. Orn/put_carbamltrans.
[Graphical view]
PfamiPF00185. OTCace. 1 hit.
PF02729. OTCace_N. 1 hit.
[Graphical view]
PRINTSiPR00100. AOTCASE.
PR00102. OTCASE.
SUPFAMiSSF53671. SSF53671. 1 hit.
TIGRFAMsiTIGR00658. orni_carb_tr. 1 hit.
PROSITEiPS00097. CARBAMOYLTRANSFERASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiOTC_MOUSE
AccessioniPrimary (citable) accession number: P11725
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1989
Last sequence update: October 1, 1989
Last modified: November 2, 2016
This is version 157 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.