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ornithine carbamoyltransferase (OTCase, ornithine transcarbamylase, OTC)
Function:
- nitrogen metabolism; urea cycle
- catalyzes condensation of ornithine with carbamoyl phosphate to form citrulline in urea cycle
carbamoyl phosphate + L-ornithine phosphate + L-citrulline
Structure:
- homotrimer
- belongs to the ATCase/OTCase family
Compartment: mitochondrial matrix
Expression: mainly in liver & intestinal mucosa
Pathology:
- defects in OTC are the cause of ornithine hyperammonemia
Interactions
molecular events
Related
urea cycle
General
mitochondrial protein
transferase
urea cycle enzyme
Properties
SIZE: entity length = 354 aa
MW = 40 kD
COMPARTMENT: mitochondria
MOTIF: binding site
SITE: 90-94
FOR-BINDING-OF: carbamoyl
ornithine
binding site
SITE: 141-141
FOR-BINDING-OF: carbamoyl
ornithine
binding site
SITE: 168-171
FOR-BINDING-OF: carbamoyl
ornithine
binding site
SITE: 199-199
FOR-BINDING-OF: ornithine
binding site
SITE: 263-267
FOR-BINDING-OF: ornithine
binding site
SITE: 302-305
FOR-BINDING-OF: ornithine
MOTIF: cysteine residue {C303}
binding site
SITE: 330-330
FOR-BINDING-OF: carbamoyl
ornithine
Database Correlations
OMIM correlations
MORBIDMAP 300461
UniProt P00480
PFAM correlations
Kegg hsa:5009
ENZYME 2.1.3.3
References
- UniProt :accession P00480
- OTC, OTCase website
http://63.75.201.100/otc/index.asp
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=OTC