P11725 · OTC_MOUSE

  • Protein
    Ornithine transcarbamylase, mitochondrial
  • Gene
    Otc
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline. The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion.

Catalytic activity

Activity regulation

Negatively regulated by lysine acetylation.

Pathway

Nitrogen metabolism; urea cycle; L-citrulline from L-ornithine and carbamoyl phosphate: step 1/1.

Features

Showing features for binding site, active site.

TypeIDPosition(s)Description
Binding site90-94carbamoyl phosphate (UniProtKB | ChEBI)
Binding site141carbamoyl phosphate (UniProtKB | ChEBI)
Binding site141L-ornithine (UniProtKB | ChEBI)
Binding site168carbamoyl phosphate (UniProtKB | ChEBI)
Binding site199L-ornithine (UniProtKB | ChEBI)
Binding site263-267L-ornithine (UniProtKB | ChEBI)
Binding site302-305L-ornithine (UniProtKB | ChEBI)
Active site303
Binding site330carbamoyl phosphate (UniProtKB | ChEBI)
Binding site330L-ornithine (UniProtKB | ChEBI)

GO annotations

AspectTerm
Cellular Componentmitochondrial inner membrane
Cellular Componentmitochondrial matrix
Cellular Componentmitochondrion
Molecular Functionamino acid binding
Molecular Functionidentical protein binding
Molecular Functionornithine carbamoyltransferase activity
Molecular Functionphosphate ion binding
Molecular Functionphospholipid binding
Biological Processammonium homeostasis
Biological Processarginine biosynthetic process via ornithine
Biological Processcitrulline biosynthetic process
Biological Processliver development
Biological Processmidgut development
Biological Processmonoatomic anion homeostasis
Biological Processornithine catabolic process
Biological Processornithine metabolic process
Biological Processresponse to biotin
Biological Processresponse to insulin
Biological Processresponse to xenobiotic stimulus
Biological Processresponse to zinc ion
Biological Processurea cycle

Keywords

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    Ornithine transcarbamylase, mitochondrial
  • EC number
  • Short names
    OTCase
  • Alternative names
    • Ornithine carbamoyltransferase, mitochondrial

Gene names

    • Name
      Otc

Organism names

  • Taxonomic identifier
  • Strain
    • C57BL/6J
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus

Accessions

  • Primary accession
    P11725

Proteomes

Organism-specific databases

Subcellular Location

Phenotypes & Variants

Involvement in disease

  • 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

Features

Showing features for natural variant.

TypeIDPosition(s)Description
Natural variant117in spf

Variants

We now provide the "Disease & Variants" viewer in its own tab.

The viewer provides 14 variants from UniProt as well as other sources including ClinVar and dbSNP.

Go to variant viewer

Keywords

PTM/Processing

Features

Showing features for transit peptide, chain, modified residue.

TypeIDPosition(s)Description
Transit peptide1-32Mitochondrion
ChainPRO_000002033533-354Ornithine transcarbamylase, mitochondrial
Modified residue70N6-acetyllysine; alternate
Modified residue70N6-succinyllysine; alternate
Modified residue80N6-succinyllysine
Modified residue88N6-acetyllysine; alternate
Modified residue88N6-succinyllysine; alternate
Modified residue133Phosphoserine
Modified residue144N6-acetyllysine; alternate
Modified residue144N6-succinyllysine; alternate
Modified residue221N6-acetyllysine; alternate
Modified residue221N6-succinyllysine; alternate
Modified residue231N6-acetyllysine; alternate
Modified residue231N6-succinyllysine; alternate
Modified residue238N6-acetyllysine; alternate
Modified residue238N6-succinyllysine; alternate
Modified residue243N6-acetyllysine
Modified residue274N6-succinyllysine
Modified residue289N6-succinyllysine
Modified residue292N6-acetyllysine; alternate
Modified residue292N6-succinyllysine; alternate
Modified residue307N6-acetyllysine; alternate
Modified residue307N6-succinyllysine; alternate

Post-translational modification

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

Keywords

Proteomic databases

PTM databases

Expression

Gene expression databases

Interaction

Subunit

Homotrimer.

Protein-protein interaction databases

Miscellaneous

Structure

Family & Domains

Sequence similarities

Keywords

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Sequence processing
    The displayed sequence is further processed into a mature form.
  • Length
    354
  • Mass (Da)
    39,765
  • Last updated
    1989-10-01 v1
  • Checksum
    33BBE5D1E88AA196
MLSNLRILLNNAALRKGHTSVVRHFWCGKPVQSQVQLKGRDLLTLKNFTGEEIQYMLWLSADLKFRIKQKGEYLPLLQGKSLGMIFEKRSTRTRLSTETGFALLGGHPSFLTTQDIHLGVNESLTDTARVLSSMTDAVLARVYKQSDLDTLAKEASIPIVNGLSDLYHPIQILADYLTLQEHYGSLKGLTLSWIGDGNNILHSIMMSAAKFGMHLQAATPKGYEPDPNIVKLAEQYAKENGTKLSMTNDPLEAARGGNVLITDTWISMGQEDEKKKRLQAFQGYQVTMKTAKVAASDWTFLHCLPRKPEEVDDEVFYSPRSLVFPEAENRKWTIMAVMVSLLTDYSPVLQKPKF

Computationally mapped potential isoform sequences

There is 1 potential isoform mapped to this entry

View all
EntryEntry nameGene nameLength
Q8R1A8Q8R1A8_MOUSEOtc351

Features

Showing features for sequence conflict.

TypeIDPosition(s)Description
Sequence conflict195in Ref. 2; CAA30121

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
M17030
EMBL· GenBank· DDBJ
AAA39865.1
EMBL· GenBank· DDBJ
mRNA
M12716
EMBL· GenBank· DDBJ
AAA39864.1
EMBL· GenBank· DDBJ
Genomic DNA Sequence problems.
X07092
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07093
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07094
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07095
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07096
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07097
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07098
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07099
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA
X07100
EMBL· GenBank· DDBJ
CAA30121.1
EMBL· GenBank· DDBJ
Genomic DNA

Genome annotation databases

Similar Proteins

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