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Details on Person UniProt:P00480 OTC

Class:Id	ReferenceGeneProduct:61024
_chainChangeLog	transit peptide:1-32 added on Sat February 7 2015;chain:33-354 added on Sat February 7 2015
_displayName	UniProt:P00480 OTC
_timestamp	2026-02-20 21:32:53
chain	transit peptide:1-32 chain:33-354
checksum	AE15B734F6E27A3B
comment	FUNCTION Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline (PubMed:2556444, PubMed:6372096, PubMed:8112735). The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion (PubMed:2556444).CATALYTIC ACTIVITY carbamoyl phosphate + L-ornithine = L-citrulline + phosphate + H(+)ACTIVITY REGULATION Negatively regulated by lysine acetylation.PATHWAY Nitrogen metabolism; urea cycle; L-citrulline from L-ornithine and carbamoyl phosphate: step 1/1.SUBUNIT Homotrimer.SUBCELLULAR LOCATION Mainly expressed in liver and intestinal mucosa.PTM Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the aspartate/ornithine carbamoyltransferase superfamily. OTCase family.
description	recommendedName: fullName evidence="59 60"Ornithine transcarbamylase, mitochondrial shortName evidence="56"OTCase ecNumber evidence="19 23 32"2.1.3.3 alternativeName: Ornithine carbamoyltransferase, mitochondrial
geneName	OTC
identifier	P00480
isSequenceChanged	FALSE
keyword	3D-structure Acetylation Amino-acid biosynthesis Arginine biosynthesis Disease variant Mitochondrion Phosphoprotein Proteomics identification Reference proteome Transferase Transit peptide Urea cycle
modified	[InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20
name	OTC
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:8989703] ENSEMBL:ENSG00000036473 OTC [Homo sapiens]
secondaryIdentifier	OTC_HUMAN A8K9P2 D3DWB0 Q3KNR1 Q6B0I1 Q9NYJ5
sequenceLength	354
species	[Species:48887] Homo sapiens
(referenceEntity)	[EntityWithAccessionedSequence:70557] OTC [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:8986136] OTC(1-354) [mitochondrial intermembrane space] [Homo sapiens] [EntityWithAccessionedSequence:8986137] OTC(1-32) [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:8986146] OTC(1-354) [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:8986170] OTC [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:9956406] OTC precursor R26Q [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:9956408] OTC precursor [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:9956411] OTC D126G [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9956412] OTC G195R [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9956413] OTC T264A [mitochondrial matrix] [Homo sapiens] List all 110 refering instances
(referenceSequence)	[ReplacedResidue:9956375] L-arginine 277 replaced with L-glutamine [ReplacedResidue:9956376] L-aspartic acid 126 replaced with glycine [ReplacedResidue:9956377] L-proline 245 replaced with L-tryptophan [NonsenseMutation:9956378] Nonsense mutation at glycine 50 [ReplacedResidue:9956380] L-arginine 129 replaced with L-histidine [ReplacedResidue:9956381] L-leucine 148 replaced with L-phenylalanine [ReplacedResidue:9956382] L-proline 225 replaced with L-arginine [ReplacedResidue:9956383] L-isoleucine 172 replaced with L-methionine [ReplacedResidue:9956384] L-leucine 45 replaced with L-proline [ReplacedResidue:9956385] L-aspartic acid 196 replaced with L-valine List all 105 refering instances
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No pathways have been reviewed or authored by UniProt:P00480 OTC (61024)