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Protein

Ornithine carbamoyltransferase, mitochondrial

Gene

Otc

Organism
Rattus norvegicus (Rat)
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

  • amino acid binding Source: RGD
  • ornithine carbamoyltransferase activity Source: RGD
  • phosphate ion binding Source: RGD
  • phospholipid binding Source: RGD

GO - Biological processi

  • ammonia homeostasis Source: Ensembl
  • anion homeostasis Source: RGD
  • arginine biosynthetic process via ornithine Source: GO_Central
  • citrulline biosynthetic process Source: RGD
  • liver development Source: RGD
  • midgut development Source: RGD
  • ornithine catabolic process Source: Ensembl
  • ornithine metabolic process Source: RGD
  • protein homotrimerization Source: RGD
  • response to biotin Source: RGD
  • response to drug Source: RGD
  • response to insulin Source: RGD
  • response to nutrient levels Source: RGD
  • response to zinc ion Source: RGD
  • urea cycle Source: RGD
Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Amino-acid biosynthesis, Arginine biosynthesis, Urea cycle

Enzyme and pathway databases

ReactomeiR-RNO-70635. Urea cycle.
SABIO-RKP00481.
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
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome X

Organism-specific databases

RGDi3236. Otc.

Subcellular locationi

GO - Cellular componenti

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

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi92R → L: Strong decrease in activity. 1 Publication1
Mutagenesisi303C → S: Increases Km for ornithine 5-fold and decreases Kcat 20-fold. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 32Mitochondrion1 PublicationAdd BLAST32
ChainiPRO_000002033933 – 354Ornithine carbamoyltransferase, mitochondrialAdd BLAST322

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei70N6-acetyllysine; alternateBy similarity1
Modified residuei70N6-succinyllysine; alternateBy similarity1
Modified residuei80N6-succinyllysineBy similarity1
Modified residuei88N6-acetyllysine; alternateBy similarity1
Modified residuei88N6-succinyllysine; alternateBy similarity1
Modified residuei133PhosphoserineBy similarity1
Modified residuei144N6-acetyllysine; alternateBy similarity1
Modified residuei144N6-succinyllysine; alternateBy similarity1
Modified residuei221N6-acetyllysine; alternateBy similarity1
Modified residuei221N6-succinyllysine; alternateBy similarity1
Modified residuei231N6-acetyllysine; alternateBy similarity1
Modified residuei231N6-succinyllysine; alternateBy similarity1
Modified residuei238N6-acetyllysine; alternateBy similarity1
Modified residuei238N6-succinyllysine; alternateBy similarity1
Modified residuei274N6-succinyllysineBy similarity1
Modified residuei289N6-succinyllysineBy similarity1
Modified residuei292N6-acetyllysine; alternateBy similarity1
Modified residuei292N6-succinyllysine; alternateBy similarity1
Modified residuei307N6-acetyllysine; alternateBy similarity1
Modified residuei307N6-succinyllysine; alternateBy similarity1

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

PaxDbiP00481.
PRIDEiP00481.

PTM databases

iPTMnetiP00481.
PhosphoSitePlusiP00481.

Expressioni

Gene expression databases

BgeeiENSRNOG00000003370.
GenevisibleiP00481. RN.

Interactioni

Subunit structurei

Homotrimer.

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000004686.

Structurei

3D structure databases

ProteinModelPortaliP00481.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni90 – 94Ornithine and carbamoyl phosphate bindingBy similarity5
Regioni168 – 171Ornithine and carbamoyl phosphate bindingBy similarity4
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.
InParanoidiP00481.
KOiK00611.
OMAiEHPCQIL.
OrthoDBiEOG091G0QB1.
PhylomeDBiP00481.
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.

P00481-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLSNLRILLN KAALRKAHTS MVRNFRYGKP VQSQVQLKGR DLLTLKNFTG
60 70 80 90 100
EEIQYMLWLS ADLKFRIKQK GEYLPLLQGK SLGMIFEKRS TRTRLSTETG
110 120 130 140 150
FALLGGHPSF LTTQDIHLGV NESLTDTARV LSSMTDAVLA RVYKQSDLDI
160 170 180 190 200
LAKEATIPIV NGLSDLYHPI QILADYLTLQ EHYGSLKGLT LSWIGDGNNI
210 220 230 240 250
LHSIMMSAAK FGMHLQAATP KGYEPDPNIV KLAEQYAKEN GTRLSMTNDP
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):39,886
Last modified:July 21, 1986 - v1
Checksum:i156B511AF7063F0C
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti39G → P in AAA41772 (PubMed:3839075).Curated1
Sequence conflicti241G → S in CAA26007 (PubMed:3839075).Curated1
Sequence conflicti241G → S in AAA41772 (PubMed:3839075).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
K03040 mRNA. Translation: AAA41768.1.
M16933
, M16924, M16925, M16926, M16928, M16929, M16930, M16932 Genomic DNA. Translation: AAA41769.1.
X01976 mRNA. Translation: CAA26007.1.
K00001 mRNA. Translation: AAA41772.1.
M11266 mRNA. Translation: AAA41767.1.
X01178 mRNA. Translation: CAA25618.1.
PIRiA00563. OWRT.
RefSeqiNP_037210.1. NM_013078.1.
UniGeneiRn.2391.

Genome annotation databases

EnsembliENSRNOT00000004686; ENSRNOP00000004686; ENSRNOG00000003370.
GeneIDi25611.
KEGGirno:25611.
UCSCiRGD:3236. rat.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
K03040 mRNA. Translation: AAA41768.1.
M16933
, M16924, M16925, M16926, M16928, M16929, M16930, M16932 Genomic DNA. Translation: AAA41769.1.
X01976 mRNA. Translation: CAA26007.1.
K00001 mRNA. Translation: AAA41772.1.
M11266 mRNA. Translation: AAA41767.1.
X01178 mRNA. Translation: CAA25618.1.
PIRiA00563. OWRT.
RefSeqiNP_037210.1. NM_013078.1.
UniGeneiRn.2391.

3D structure databases

ProteinModelPortaliP00481.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000004686.

PTM databases

iPTMnetiP00481.
PhosphoSitePlusiP00481.

Proteomic databases

PaxDbiP00481.
PRIDEiP00481.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSRNOT00000004686; ENSRNOP00000004686; ENSRNOG00000003370.
GeneIDi25611.
KEGGirno:25611.
UCSCiRGD:3236. rat.

Organism-specific databases

CTDi5009.
RGDi3236. Otc.

Phylogenomic databases

eggNOGiKOG1504. Eukaryota.
COG0078. LUCA.
GeneTreeiENSGT00510000047417.
HOGENOMiHOG000022686.
HOVERGENiHBG007881.
InParanoidiP00481.
KOiK00611.
OMAiEHPCQIL.
OrthoDBiEOG091G0QB1.
PhylomeDBiP00481.
TreeFamiTF352580.

Enzyme and pathway databases

UniPathwayiUPA00158; UER00271.
ReactomeiR-RNO-70635. Urea cycle.
SABIO-RKP00481.

Miscellaneous databases

PROiP00481.

Gene expression databases

BgeeiENSRNOG00000003370.
GenevisibleiP00481. RN.

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_RAT
AccessioniPrimary (citable) accession number: P00481
Secondary accession number(s): Q63407
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: July 21, 1986
Last modified: November 2, 2016
This is version 151 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, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. 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.