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Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)

 OTC_CHICK               Reviewed;         354 AA.
Q9YHY9;
15-AUG-2003, integrated into UniProtKB/Swiss-Prot.
01-MAY-1999, sequence version 1.
28-FEB-2018, entry version 106.
RecName: Full=Ornithine carbamoyltransferase, mitochondrial;
EC=2.1.3.3;
AltName: Full=Ornithine transcarbamylase;
Short=OTCase;
Flags: Precursor;
Name=OTC;
Gallus gallus (Chicken).
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
Archelosauria; Archosauria; Dinosauria; Saurischia; Theropoda;
Coelurosauria; Aves; Neognathae; Galloanserae; Galliformes;
Phasianidae; Phasianinae; Gallus.
NCBI_TaxID=9031;
[1]
NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA].
STRAIN=White leghorn {ECO:0000269|PubMed:9792920};
TISSUE=Kidney {ECO:0000269|PubMed:9792920};
PubMed=9792920; DOI=10.1093/oxfordjournals.jbchem.a022214;
Shimogiri T., Kono M., Mannen H., Mizutani M., Tsuji S.;
"Chicken ornithine transcarbamylase gene, structure, regulation, and
chromosomal assignment: repetitive sequence motif in intron 3
regulates this enzyme activity.";
J. Biochem. 124:962-971(1998).
[2] {ECO:0000305}
ENZYME ACTIVITY.
PubMed=7332529; DOI=10.1007/BF00504253;
Tsuji S., Fukushima T.;
"Comparison of renal ornithine transcarbamylase activities within
different chicken breeds.";
Biochem. Genet. 19:881-893(1981).
[3] {ECO:0000305}
ENZYME KINETICS.
PubMed=6419722; DOI=10.1007/BF00483945;
Tsuji S., Nakagawa K., Fukushima T.;
"Genetically controlled quantitative variation of ornithine
transcarbamylase in the chick kidney.";
Biochem. Genet. 21:857-869(1983).
[4] {ECO:0000305}
GENETIC REGULATION.
PubMed=6661175; DOI=10.1007/BF00483944;
Tsuji S., Nakagawa K., Fukushima T.;
"Genetic control of ornithine transcarbamylase induction in chick
kidney.";
Biochem. Genet. 21:843-855(1983).
[5] {ECO:0000305}
ENZYME KINETICS, AND SUBUNIT.
PubMed=6418730;
Tsuji S.;
"Chicken ornithine transcarbamylase: purification and some
properties.";
J. Biochem. 94:1307-1315(1983).
[6] {ECO:0000305}
INDUCTION, AND DEVELOPMENTAL STAGE.
PubMed=6682551; DOI=10.3382/ps.0620519;
Tsuji S., Nakagawa K., Nomura Y., Mukai F., Fukushima T.;
"Induction of ornithine transcarbamylase activity with egg yolk in
chick kidney.";
Poult. Sci. 62:519-524(1983).
[7] {ECO:0000305}
TISSUE SPECIFICITY.
PubMed=3606562; DOI=10.1007/BF00499319;
Tsuji S., Kanazawa S.;
"Chicken ornithine transcarbamylase: its unexpected expression.";
Biochem. Genet. 25:259-266(1987).
-!- CATALYTIC ACTIVITY: Carbamoyl phosphate + L-ornithine = phosphate
+ L-citrulline. {ECO:0000250|UniProtKB:P00480,
ECO:0000269|PubMed:7332529}.
-!- ENZYME REGULATION: Inhibition by ornithine increases at higher pH.
{ECO:0000269|PubMed:6418730}.
-!- BIOPHYSICOCHEMICAL PROPERTIES:
Kinetic parameters:
KM=0.11 mM for carbamyl phosphate (at pH 7.5);
KM=1.21 mM for ornithine (at pH 7.5);
Note=With an increase in pH, a decrease in KM values for
ornithine and an increase in the extent of inhibition by
ornithine are observed.;
pH dependence:
Optimum pH is 7.5 in the presence of 5 mM ornithine. The curve
shifts toward a more alkaline region with a decrease in
ornithine concentration.;
-!- PATHWAY: Nitrogen metabolism; urea cycle; L-citrulline from L-
ornithine and carbamoyl phosphate: step 1/1.
-!- SUBUNIT: Homotrimer. {ECO:0000269|PubMed:6418730}.
-!- SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250}.
-!- TISSUE SPECIFICITY: Expressed in kidney, brain, heart, liver,
pancreas, gizzard, small intestine and breast muscle. More
abundant in mitochondrion-rich organs (heart, liver and brain)
than in other organs. Activity is only detected in the kidney.
{ECO:0000269|PubMed:3606562}.
-!- DEVELOPMENTAL STAGE: Activity detectable in embryos by day 14.
Increases until 7 days post-hatching, then decreases again.
{ECO:0000269|PubMed:6682551}.
-!- INDUCTION: By diet of egg yolk in animals which have a high level
of OTC activity due to presence of OCB gene.
{ECO:0000269|PubMed:6682551}.
-!- PTM: Cleavage of the precursor form to the active form occurs only
in the kidney. {ECO:0000269|PubMed:3606562}.
-!- MISCELLANEOUS: Ornithine transcarbamylase activity varies within
and between different breeds of chicken.
-!- SIMILARITY: Belongs to the ATCase/OTCase family. {ECO:0000305}.
-----------------------------------------------------------------------
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EMBL; AF065629; AAD12234.1; -; mRNA.
EMBL; AF065638; AAD33083.1; -; Genomic_DNA.
EMBL; AF065630; AAD33083.1; JOINED; Genomic_DNA.
EMBL; AF065631; AAD33083.1; JOINED; Genomic_DNA.
EMBL; AF065632; AAD33083.1; JOINED; Genomic_DNA.
EMBL; AF065634; AAD33083.1; JOINED; Genomic_DNA.
EMBL; AF065635; AAD33083.1; JOINED; Genomic_DNA.
EMBL; AF065636; AAD33083.1; JOINED; Genomic_DNA.
EMBL; AF065637; AAD33083.1; JOINED; Genomic_DNA.
PIR; JE0309; JE0309.
RefSeq; NP_990241.1; NM_204910.1.
UniGene; Gga.434; -.
ProteinModelPortal; Q9YHY9; -.
SMR; Q9YHY9; -.
STRING; 9031.ENSGALP00000026160; -.
PaxDb; Q9YHY9; -.
PRIDE; Q9YHY9; -.
GeneID; 395735; -.
KEGG; gga:395735; -.
CTD; 5009; -.
eggNOG; KOG1504; Eukaryota.
eggNOG; COG0078; LUCA.
HOGENOM; HOG000022686; -.
HOVERGEN; HBG007881; -.
InParanoid; Q9YHY9; -.
KO; K00611; -.
PhylomeDB; Q9YHY9; -.
Reactome; R-GGA-187630; Arginine metabolism.
SABIO-RK; Q9YHY9; -.
UniPathway; UPA00158; UER00271.
PRO; PR:Q9YHY9; -.
Proteomes; UP000000539; Unplaced.
GO; GO:0005759; C:mitochondrial matrix; TAS:Reactome.
GO; GO:0005739; C:mitochondrion; IBA:GO_Central.
GO; GO:0016597; F:amino acid binding; IEA:InterPro.
GO; GO:0004585; F:ornithine carbamoyltransferase activity; IDA:UniProtKB.
GO; GO:0042450; P:arginine biosynthetic process via ornithine; IBA:GO_Central.
GO; GO:0006525; P:arginine metabolic process; TAS:Reactome.
GO; GO:0000050; P:urea cycle; IBA:GO_Central.
Gene3D; 3.40.50.1370; -; 3.
InterPro; IPR006132; Asp/Orn_carbamoyltranf_P-bd.
InterPro; IPR006130; Asp/Orn_carbamoylTrfase.
InterPro; IPR036901; Asp/Orn_carbamoylTrfase_sf.
InterPro; IPR006131; Asp_carbamoyltransf_Asp/Orn-bd.
InterPro; IPR002292; Orn/put_carbamltrans.
Pfam; PF00185; OTCace; 1.
Pfam; PF02729; OTCace_N; 1.
PRINTS; PR00100; AOTCASE.
PRINTS; PR00102; OTCASE.
SUPFAM; SSF53671; SSF53671; 1.
TIGRFAMs; TIGR00658; orni_carb_tr; 1.
PROSITE; PS00097; CARBAMOYLTRANSFERASE; 1.
1: Evidence at protein level;
Amino-acid biosynthesis; Arginine biosynthesis; Complete proteome;
Mitochondrion; Reference proteome; Transferase; Transit peptide;
Urea cycle.
TRANSIT 1 32 Mitochondrion. {ECO:0000250}.
CHAIN 33 354 Ornithine carbamoyltransferase,
mitochondrial.
/FTId=PRO_0000020337.
REGION 90 94 Carbamoyl phosphate binding.
{ECO:0000250}.
REGION 263 267 Ornithine binding. {ECO:0000250}.
REGION 302 305 Ornithine binding. {ECO:0000250}.
ACT_SITE 303 303 {ECO:0000250}.
BINDING 141 141 Carbamoyl phosphate. {ECO:0000250}.
BINDING 141 141 Ornithine. {ECO:0000250}.
BINDING 168 168 Carbamoyl phosphate. {ECO:0000250}.
BINDING 199 199 Ornithine. {ECO:0000250}.
BINDING 330 330 Carbamoyl phosphate. {ECO:0000250}.
BINDING 330 330 Ornithine. {ECO:0000250}.
SEQUENCE 354 AA; 40245 MW; 20447180BAD9D4ED CRC64;
MLFNLKNLYR ITKLTQNSKH LPRHFCRGPP NQMNVCLKGR DLLTLQNYTA DELKYLLWVA
SDLKQRIKDK GEYLPLMQGK SLAMIFEKRS TRTRLSAETG FALLGGHSSF LTKQDIHLGT
NESLTDTARV LSSMTNAILA RVYKHNDLDL MTKEATIPVI NGLSDLYHPL QILADYLTLQ
EHYGGLNGLT IAWIGDGNNV LHSIMTSAAK LGMHLRIATP KGFEPDLRIT KVTEQYSKEY
GTRLLLTTDP LEAANGANVL VTDTWISMGQ EEEKRRRLKA FQGYQITMQT VQSAASNWTF
LHCLPRKPEE VDDEVFYSPR SLVFQEAENR KWTIMAVMVS LLTDYSPQLQ MPTF


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