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P11725

- OTC_MOUSE

UniProt

P11725 - OTC_MOUSE

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Protein
Ornithine carbamoyltransferase, mitochondrial
Gene
Otc
Organism
Mus musculus (Mouse)
Status
Reviewed - 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

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei141 – 1411Carbamoyl phosphate By similarity
Binding sitei141 – 1411Ornithine By similarity
Binding sitei168 – 1681Carbamoyl phosphate By similarity
Binding sitei199 – 1991Ornithine By similarity
Active sitei303 – 3031 By similarity
Binding sitei330 – 3301Carbamoyl phosphate By similarity
Binding sitei330 – 3301Ornithine By similarity

GO - Molecular functioni

  1. amino acid binding Source: Ensembl
  2. ornithine carbamoyltransferase activity Source: UniProtKB-EC
  3. phosphate ion binding Source: Ensembl
  4. phospholipid binding Source: Ensembl

GO - Biological processi

  1. anion homeostasis Source: Ensembl
  2. arginine biosynthetic process Source: UniProtKB-KW
  3. citrulline biosynthetic process Source: Ensembl
  4. liver development Source: Ensembl
  5. midgut development Source: Ensembl
  6. protein homotrimerization Source: Ensembl
  7. response to biotin Source: Ensembl
  8. response to drug Source: Ensembl
  9. response to insulin Source: Ensembl
  10. response to zinc ion Source: Ensembl
  11. urea cycle Source: UniProtKB-UniPathway
Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Amino-acid biosynthesis, Arginine biosynthesis, Urea cycle

Enzyme and pathway databases

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
ProteomesiUP000000589: Chromosome X

Organism-specific databases

MGIiMGI:97448. Otc.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: MGI
  2. mitochondrial matrix Source: UniProtKB-SubCell
  3. 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)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3232Mitochondrion
Add
BLAST
Chaini33 – 354322Ornithine carbamoyltransferase, mitochondrial
PRO_0000020335Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei70 – 701N6-acetyllysine; alternate1 Publication
Modified residuei70 – 701N6-succinyllysine; alternate1 Publication
Modified residuei80 – 801N6-succinyllysine1 Publication
Modified residuei88 – 881N6-acetyllysine; alternate1 Publication
Modified residuei88 – 881N6-succinyllysine; alternate1 Publication
Modified residuei144 – 1441N6-acetyllysine; alternate1 Publication
Modified residuei144 – 1441N6-succinyllysine; alternate1 Publication
Modified residuei221 – 2211N6-acetyllysine; alternate1 Publication
Modified residuei221 – 2211N6-succinyllysine; alternate1 Publication
Modified residuei231 – 2311N6-acetyllysine; alternate1 Publication
Modified residuei231 – 2311N6-succinyllysine; alternate1 Publication
Modified residuei238 – 2381N6-acetyllysine; alternate1 Publication
Modified residuei238 – 2381N6-succinyllysine; alternate1 Publication
Modified residuei243 – 2431N6-acetyllysine1 Publication
Modified residuei274 – 2741N6-succinyllysine1 Publication
Modified residuei289 – 2891N6-succinyllysine1 Publication
Modified residuei292 – 2921N6-acetyllysine; alternate1 Publication
Modified residuei292 – 2921N6-succinyllysine; alternate1 Publication
Modified residuei307 – 3071N6-acetyllysine; alternate1 Publication
Modified residuei307 – 3071N6-succinyllysine; alternate1 Publication

Post-translational modificationi

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

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiP11725.
PaxDbiP11725.
PRIDEiP11725.

2D gel databases

SWISS-2DPAGEP11725.

PTM databases

PhosphoSiteiP11725.

Expressioni

Gene expression databases

ArrayExpressiP11725.
BgeeiP11725.
CleanExiMM_OTC.
GenevestigatoriP11725.

Interactioni

Subunit structurei

Homotrimer.

Protein-protein interaction databases

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

Structurei

3D structure databases

ProteinModelPortaliP11725.
SMRiP11725. Positions 34-354.

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni90 – 945Carbamoyl phosphate binding By similarity
Regioni263 – 2675Ornithine binding By similarity
Regioni302 – 3054Ornithine binding By similarity

Sequence similaritiesi

Belongs to the ATCase/OTCase family.

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0078.
HOGENOMiHOG000022686.
HOVERGENiHBG007881.
InParanoidiP11725.
KOiK00611.
OMAiKWAEQNA.
OrthoDBiEOG7D85XC.
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

MLSNLRILLN NAALRKGHTS VVRHFWCGKP VQSQVQLKGR DLLTLKNFTG    50
EEIQYMLWLS ADLKFRIKQK GEYLPLLQGK SLGMIFEKRS TRTRLSTETG 100
FALLGGHPSF LTTQDIHLGV NESLTDTARV LSSMTDAVLA RVYKQSDLDT 150
LAKEASIPIV NGLSDLYHPI QILADYLTLQ EHYGSLKGLT LSWIGDGNNI 200
LHSIMMSAAK FGMHLQAATP KGYEPDPNIV KLAEQYAKEN GTKLSMTNDP 250
LEAARGGNVL ITDTWISMGQ EDEKKKRLQA FQGYQVTMKT AKVAASDWTF 300
LHCLPRKPEE VDDEVFYSPR SLVFPEAENR KWTIMAVMVS LLTDYSPVLQ 350
KPKF 354
Length:354
Mass (Da):39,765
Last modified:October 1, 1989 - v1
Checksum:i33BBE5D1E88AA196
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti117 – 1171H → N in spf. 1 Publication

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti195 – 1951G → R in CAA30121. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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_008769.3.
UniGeneiMm.2611.

Genome annotation databases

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

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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 .
CCDSi CCDS30015.1.
PIRi A43609. OWMS.
RefSeqi NP_032795.1. NM_008769.3.
UniGenei Mm.2611.

3D structure databases

ProteinModelPortali P11725.
SMRi P11725. Positions 34-354.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

IntActi P11725. 2 interactions.
MINTi MINT-1838977.
STRINGi 10090.ENSMUSP00000056152.

PTM databases

PhosphoSitei P11725.

2D gel databases

SWISS-2DPAGE P11725.

Proteomic databases

MaxQBi P11725.
PaxDbi P11725.
PRIDEi P11725.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000049910 ; ENSMUSP00000056152 ; ENSMUSG00000031173 .
GeneIDi 18416.
KEGGi mmu:18416.
UCSCi uc009sqk.1. mouse.

Organism-specific databases

CTDi 5009.
MGIi MGI:97448. Otc.

Phylogenomic databases

eggNOGi COG0078.
HOGENOMi HOG000022686.
HOVERGENi HBG007881.
InParanoidi P11725.
KOi K00611.
OMAi KWAEQNA.
OrthoDBi EOG7D85XC.
PhylomeDBi P11725.
TreeFami TF352580.

Enzyme and pathway databases

UniPathwayi UPA00158 ; UER00271 .

Miscellaneous databases

NextBioi 294052.
PROi P11725.
SOURCEi Search...

Gene expression databases

ArrayExpressi P11725.
Bgeei P11725.
CleanExi MM_OTC.
Genevestigatori P11725.

Family and domain databases

Gene3Di 3.40.50.1370. 2 hits.
InterProi IPR006132. Asp/Orn_carbamoyltranf_P-bd.
IPR006130. Asp/Orn_carbamoylTrfase.
IPR006131. Asp_carbamoyltransf_Asp/Orn-bd.
IPR002292. Orn/put_carbamltrans.
[Graphical view ]
Pfami PF00185. OTCace. 1 hit.
PF02729. OTCace_N. 1 hit.
[Graphical view ]
PRINTSi PR00100. AOTCASE.
PR00102. OTCASE.
SUPFAMi SSF53671. SSF53671. 1 hit.
TIGRFAMsi TIGR00658. orni_carb_tr. 1 hit.
PROSITEi PS00097. CARBAMOYLTRANSFERASE. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "The molecular basis of the sparse fur mouse mutation."
    Veres G., Gibbs R.A., Scherer S.E., Caskey C.T.
    Science 237:415-417(1987) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT ASN-117.
    Strain: C57BL/6J.
  2. "The genetic structure of mouse ornithine transcarbamylase."
    Scherer S.E., Veres G., Caskey C.T.
    Nucleic Acids Res. 16:1593-1601(1988) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
  3. "The 5' flanking region of the ornithine transcarbamylase gene contains DNA sequences regulating tissue-specific expression."
    Veres G., Craigen W.J., Caskey C.T.
    J. Biol. Chem. 261:7588-7591(1986) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-26.
    Strain: C57BL/6J.
  4. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
    Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
    Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-70; LYS-80; LYS-88; LYS-144; LYS-221; LYS-231; LYS-238; LYS-274; LYS-289; LYS-292 AND LYS-307, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  5. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
    Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
    Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-70; LYS-88; LYS-144; LYS-221; LYS-231; LYS-238; LYS-243; LYS-292 AND LYS-307, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

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: July 9, 2014
This is version 135 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

External Data

Dasty 3

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