UniProt Accession

 OTC_HUMAN; P00480; A8K9P2; D3DWB0; Q3KNR1; Q6B0I1; Q9NYJ5 

Genbank Protein ID

 K02100; D00230; AK292757; AL607040; AF241726; AL606748; AL606748; AL607040; AF241726; CH471141; CH471141; BC074745; BC107153; BC107154; BC114496; M11235; D00095; X04443; S73640 

Genbank Nucleotide ID

 AAA59975.1; BAA00161.1; BAF85446.1; CAI95193.1; CAI95193.1; CAI95193.1; CAI95408.1; CAI95408.1; CAI95408.1; EAW59439.1; EAW59440.1; AAH74745.1; AAI07154.1; AAI07155.1; AAI14497.1; AAA59976.1; BAA00058.1; CAA28039.1; AAB31859.1 

Protein Name

 Ornithine carbamoyltransferase, mitochondrial 

Protein Synonyms/Alias

 Ornithine transcarbamylase; OTCase 

Gene Name

 OTC 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
46	GRDLLTLKNFTGEEI	acetylation	[1]
88	SLGMIFEKRSTRTRL	acetylation	[2]

Reference

 [1] Regulation of cellular metabolism by protein lysine acetylation.
 Zhao S, Xu W, Jiang W, Yu W, Lin Y, Zhang T, Yao J, Zhou L, Zeng Y, Li H, Li Y, Shi J, An W, Hancock SM, He F, Qin L, Chin J, Yang P, Chen X, Lei Q, Xiong Y, Guan KL.
 Science. 2010 Feb 19;327(5968):1000-4. [PMID: 20167786]
 [2] Lysine 88 acetylation negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.
 Yu W, Lin Y, Yao J, Huang W, Lei Q, Xiong Y, Zhao S, Guan KL.
 J Biol Chem. 2009 May 15;284(20):13669-75. [PMID: 19318352] 

Functional Description

  

Sequence Annotation

 REGION 90 94 Ornithine and carbamoyl phosphate
 REGION 168 171 Ornithine and carbamoyl phosphate
 REGION 263 267 Ornithine binding.
 REGION 302 305 Ornithine binding (By similarity).
 ACT_SITE 303 303 By similarity.
 BINDING 141 141 Carbamoyl phosphate.
 BINDING 141 141 Ornithine.
 BINDING 199 199 Ornithine.
 BINDING 330 330 Carbamoyl phosphate.
 BINDING 330 330 Ornithine.
 MOD_RES 88 88 N6-acetyllysine.
 MOD_RES 231 231 N6-acetyllysine (By similarity).
 MOD_RES 238 238 N6-acetyllysine (By similarity).  

Keyword

 3D-structure; Acetylation; Amino-acid biosynthesis; Arginine biosynthesis; Complete proteome; Disease mutation; Mitochondrion; Polymorphism; Reference proteome; Transferase; Transit peptide; Urea cycle. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 354 AA 

Protein Sequence

Gene Ontology

 GO:0005743; C:mitochondrial inner membrane; IEA:Compara.
 GO:0005759; C:mitochondrial matrix; NAS:UniProtKB.
 GO:0009348; C:ornithine carbamoyltransferase complex; NAS:UniProtKB.
 GO:0016597; F:amino acid binding; IEA:Compara.
 GO:0004585; F:ornithine carbamoyltransferase activity; NAS:UniProtKB.
 GO:0042301; F:phosphate ion binding; IEA:Compara.
 GO:0005543; F:phospholipid binding; IEA:Compara.
 GO:0055081; P:anion homeostasis; IEA:Compara.
 GO:0006526; P:arginine biosynthetic process; NAS:UniProtKB.
 GO:0019240; P:citrulline biosynthetic process; IEA:Compara.
 GO:0001889; P:liver development; IEA:Compara.
 GO:0007494; P:midgut development; IEA:Compara.
 GO:0070207; P:protein homotrimerization; IEA:Compara.
 GO:0070781; P:response to biotin; IEA:Compara.
 GO:0042493; P:response to drug; IEA:Compara.
 GO:0032868; P:response to insulin stimulus; IEA:Compara.
 GO:0010043; P:response to zinc ion; IEA:Compara.
 GO:0000050; P:urea cycle; NAS:UniProtKB. 

Interpro

 IPR006132; Asp/Orn_carbamoyltranf_P-bd.
 IPR006130; Asp/Orn_carbamoylTrfase.
 IPR006131; Asp_carbamoyltransf_Asp/Orn-bd.
 IPR002292; Orn/put_carbamltrans. 

Pfam

 PF00185; OTCace
 PF02729; OTCace_N 

SMART

  

PROSITE

 PS00097; CARBAMOYLTRANSFERASE 

PRINTS

 PR00100; AOTCASE.
 PR00102; OTCASE.