Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Ornithine carbamoyltransferase, mitochondrial

Gene

Otc

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionTransferase
Biological processAmino-acid biosynthesis, Arginine biosynthesis, Urea cycle

Enzyme and pathway databases

ReactomeiR-MMU-1268020 Mitochondrial protein import
R-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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome X

Organism-specific databases

MGIiMGI:97448 Otc

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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.1 Publication

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
MINTiP11725
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
OMAiDGNNVCN
OrthoDBiEOG091G0QB1
PhylomeDBiP11725
TreeFamiTF352580

Family and domain databases

Gene3Di3.40.50.1370, 3 hits
InterProiView protein in InterPro
IPR006132 Asp/Orn_carbamoyltranf_P-bd
IPR006130 Asp/Orn_carbamoylTrfase
IPR036901 Asp/Orn_carbamoylTrfase_sf
IPR006131 Asp_carbamoyltransf_Asp/Orn-bd
IPR002292 Orn/put_carbamltrans
PfamiView protein in Pfam
PF00185 OTCace, 1 hit
PF02729 OTCace_N, 1 hit
PRINTSiPR00100 AOTCASE
PR00102 OTCASE
SUPFAMiSSF53671 SSF53671, 1 hit
TIGRFAMsiTIGR00658 orni_carb_tr, 1 hit
PROSITEiView protein in PROSITE
PS00097 CARBAMOYLTRANSFERASE, 1 hit

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

Similar proteinsi

Entry informationi

Entry nameiOTC_MOUSE
AccessioniPrimary (citable) accession number: P11725
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1989
Last sequence update: October 1, 1989
Last modified: May 23, 2018
This is version 166 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Cookie policy

We would like to use anonymized google analytics cookies to gather statistics on how uniprot.org is used in aggregate. Learn more

UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health