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P11725

- OTC_MOUSE

UniProt

P11725 - OTC_MOUSE

Protein

Ornithine carbamoyltransferase, mitochondrial

Gene

Otc

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 136 (01 Oct 2014)
      Sequence version 1 (01 Oct 1989)
      Previous versions | rss
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    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 phosphateBy similarity
    Binding sitei141 – 1411OrnithineBy similarity
    Binding sitei168 – 1681Carbamoyl phosphateBy similarity
    Binding sitei199 – 1991OrnithineBy similarity
    Active sitei303 – 3031By similarity
    Binding sitei330 – 3301Carbamoyl phosphateBy similarity
    Binding sitei330 – 3301OrnithineBy 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

    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

    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 – 3232MitochondrionAdd
    BLAST
    Chaini33 – 354322Ornithine carbamoyltransferase, mitochondrialPRO_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.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Region

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

    Sequence similaritiesi

    Belongs to the ATCase/OTCase family.Curated

    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

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti195 – 1951G → R in CAA30121. (PubMed:2831503)Curated

    Natural variant

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

    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: October 1, 2014
    This is version 136 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