General Information of Drug Therapeutic Target (DTT) (ID: TT5KIO9)

DTT Name Ornithine transcarbamylase (OTC)
Synonyms OTCase; Ornithine carbamoyltransferase, mitochondrial
Gene Name OTC
DTT Type
Clinical trial target
[1]
UniProt ID
OTC_HUMAN
TTD ID
T52098
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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EC Number
EC 2.1.3.3
Sequence
MLFNLRILLNNAAFRNGHNFMVRNFRCGQPLQNKVQLKGRDLLTLKNFTGEEIKYMLWLS
ADLKFRIKQKGEYLPLLQGKSLGMIFEKRSTRTRLSTETGFALLGGHPCFLTTQDIHLGV
NESLTDTARVLSSMADAVLARVYKQSDLDTLAKEASIPIINGLSDLYHPIQILADYLTLQ
EHYSSLKGLTLSWIGDGNNILHSIMMSAAKFGMHLQAATPKGYEPDASVTKLAEQYAKEN
GTKLLLTNDPLEAAHGGNVLITDTWISMGQEEEKKKRLQAFQGYQVTMKTAKVAASDWTF
LHCLPRKPEEVDDEVFYSPRSLVFPEAENRKWTIMAVMVSLLTDYSPQLQKPKF
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.
KEGG Pathway
Arginine biosynthesis (hsa00220 )
Metabolic pathways (hsa01100 )
Biosynthesis of amino acids (hsa01230 )
Reactome Pathway
Urea cycle (R-HSA-70635 )
Mitochondrial protein import (R-HSA-1268020 )
BioCyc Pathway
MetaCyc:HS00516-MON

Molecular Interaction Atlas (MIA) of This DTT

Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DTT
3 Clinical Trial Drug(s) Targeting This DTT
Drug Name Drug ID Indication ICD 11 Highest Status REF
Avalotcagene ontaparvovec DMAOHT4 Ornithine transcarbamylase deficiency 5C50.AY Phase 3 [2]
ARCT-810 DM91F9I Ornithine transcarbamylase deficiency 5C50.AY Phase 2 [3]
DTX301 DM8M71U Ornithine transcarbamylase deficiency 5C50.AY Phase 1/2 [1]
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References

1 Clinical pipeline report, company report or official report of Ultragenyx Pharmaceutical.
2 Prednisolone reduces the interferon response to AAV in cynomolgus macaques and may increase liver gene expression. Mol Ther Methods Clin Dev. 2022 Jan 19;24:292-305.
3 Clinical pipeline report, company report or official report of Arcturus Therapeutics