Gene Information
Gene NameOTC
OrganismHomo sapiens (Human)
Gene Length354
Protein NamesOrnithine transcarbamylase, mitochondrial (OTCase) (EC 2.1.3.3) (Ornithine carbamoyltransferase, mitochondrial)
Target Nameornithine transcarbamylase
Target TypeClinical trial target
Gene Age544 ( ENSG00000036473 )
Evolutionary StageCellular_organisms
AlphaFoldDBP00480
Gene CardOTC
Uniprot IDP00480
PfamPF00185; PF02729
In ASDno
Allosteric Predictionyes (0.578466024)
Ortholog in AnimalsAlpaca_ OTC ( ortholog_one2one )
Alpaca_ OTC ( ortholog_one2many )
Dog_ OTC ( ortholog_one2one )
GuineaPig_ OTC ( ortholog_one2one )
Macaque_ OTC ( ortholog_one2one )
Mouse_ Otc ( ortholog_one2one )
Pig_ OTC ( ortholog_one2one )
Rat_ Otc ( ortholog_one2one )
Rabbit_ OTC ( ortholog_one2one )
OhnologsNA
ParalogsCAD, CPS1
Tissueduodenum, liver, small.intestine
Tissue SpecificityMainly expressed in liver and intestinal mucosa.
Pharmacological Animal ModelsNA
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 1C9Y  (X-ray  1.90A  1EP9  (X-ray  2.40A  1FVO  (X-ray  2.60A  1OTH  (X-ray  1.85A 
DBSNP rs68031618  rs72554306  rs72554307  rs72554308  rs72554309  rs72554310  rs72554312  rs72554311  rs1800321  rs67939655  rs67486158  rs72554319  rs72554320  rs72554323  rs72554324  rs72554331  rs72554337  rs72554336  rs72554338  rs72554339  rs72554342  rs66550389  rs72554344  rs72554345  rs72554349  rs1133135  rs72554350  rs1800324  rs66539573  rs66539573  rs72554356  rs72554358  rs66656800  rs72556259  rs72556260  rs68026851  rs68026851  rs66741318  rs72556269  rs67954347  rs72556271  rs66626662  rs72556276  rs66867430  rs72556279  rs72556280  rs72556281  rs68033093  rs72556283  rs72556284  rs72556287  rs72556290  rs72556291  rs72556293  rs72556292  rs72556294  rs72556295  rs72556296  rs72556298  rs67294955  rs72556300  rs66642398  rs72556302  rs72556301  rs72558404  rs72558407  rs72558408  rs72558410  rs72558413  rs72558412  rs72558415  rs72558416  rs72558417  rs72558420  rs72558423  rs72558425  rs67120076  rs67120076  rs72558428  rs72558435  rs72558436  rs72558437  rs72558440  rs67333670  rs72558443  rs72558442  rs72558444  rs67156896  rs72558446  rs72558448  rs72558449  rs72558450  rs1800328  rs72558452  rs66724222  rs72558454  rs72558462  rs67993095  rs67870244  rs72558463  rs67468335  rs72558464  rs72558465  rs67501347  rs72558474  rs72558476  rs72558478  rs72558486  rs72558487  rs72558488  rs72558489  rs72558490  rs72558491  rs72558492  rs66469337  rs72558495 
Subcellular location [CC]Mitochondrion matrix {ECO:0000269|PubMed:3895227}.
Mouse PG classificationNA
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