ID OTC_MOUSE Reviewed; 354 AA. AC P11725; DT 01-OCT-1989, integrated into UniProtKB/Swiss-Prot. DT 01-OCT-1989, sequence version 1. DT 26-FEB-2008, entry version 79. DE Ornithine carbamoyltransferase, mitochondrial precursor (EC 2.1.3.3) DE (OTCase) (Ornithine transcarbamylase). GN Name=Otc; OS Mus musculus (Mouse). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Sciurognathi; OC Muroidea; Muridae; Murinae; Mus. OX NCBI_TaxID=10090; RN [1] RP NUCLEOTIDE SEQUENCE [MRNA], AND VARIANT ASN-117. RC STRAIN=C57BL/6J; RX MEDLINE=87263407; PubMed=3603027; RA Veres G., Gibbs R.A., Scherer S.E., Caskey C.T.; RT "The molecular basis of the sparse fur mouse mutation."; RL Science 237:415-417(1987). RN [2] RP NUCLEOTIDE SEQUENCE [GENOMIC DNA]. RX MEDLINE=88157717; PubMed=2831503; DOI=10.1093/nar/16.4.1593; RA Scherer S.E., Veres G., Caskey C.T.; RT "The genetic structure of mouse ornithine transcarbamylase."; RL Nucleic Acids Res. 16:1593-1601(1988). RN [3] RP NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-26. RC STRAIN=C57BL/6J; RX MEDLINE=86224037; PubMed=3011788; RA Veres G., Craigen W.J., Caskey C.T.; RT "The 5' flanking region of the ornithine transcarbamylase gene RT contains DNA sequences regulating tissue-specific expression."; RL J. Biol. Chem. 261:7588-7591(1986). RN [4] RP ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-88; LYS-231 AND LYS-238, AND RP MASS SPECTROMETRY. RC TISSUE=Liver; RX PubMed=16916647; DOI=10.1016/j.molcel.2006.06.026; RA Kim S.C., Sprung R., Chen Y., Xu Y., Ball H., Pei J., Cheng T., RA Kho Y., Xiao H., Xiao L., Grishin N.V., White M., Yang X.-J., Zhao Y.; RT "Substrate and functional diversity of lysine acetylation revealed by RT a proteomics survey."; RL Mol. Cell 23:607-618(2006). CC -!- CATALYTIC ACTIVITY: Carbamoyl phosphate + L-ornithine = phosphate CC + L-citrulline. CC -!- PATHWAY: Nitrogen metabolism; urea cycle; L-citrulline from L- CC ornithine and carbamoyl phosphate: step 1/1. CC -!- SUBUNIT: Homotrimer. CC -!- SUBCELLULAR LOCATION: Mitochondrion matrix. CC -!- DISEASE: Defects in Otc are the cause of the Sparse fur (spf) CC phenotype. Spf mouse have an OTCase with an overall decrease in CC activity, and altered substrate affinity. CC -!- SIMILARITY: Belongs to the ATCase/OTCase family. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; M17030; AAA39865.1; -; mRNA. DR EMBL; M12716; AAA39864.1; ALT_SEQ; Genomic_DNA. DR EMBL; X07092; CAA30121.1; -; Genomic_DNA. DR EMBL; X07093; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07094; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07095; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07096; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07097; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07098; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07099; CAA30121.1; JOINED; Genomic_DNA. DR EMBL; X07100; CAA30121.1; JOINED; Genomic_DNA. DR PIR; A43609; OWMS. DR RefSeq; NP_032795.1; -. DR UniGene; Mm.2611; -. DR HSSP; P00480; 1OTH. DR SMR; P11725; 34-354. DR SWISS-2DPAGE; P11725; -. DR Ensembl; ENSMUSG00000031173; Mus musculus. DR GeneID; 18416; -. DR KEGG; mmu:18416; -. DR MGI; MGI:97448; Otc. DR ArrayExpress; P11725; -. DR CleanEx; MM_OTC; -. DR GermOnline; ENSMUSG00000031173; Mus musculus. DR GO; GO:0005743; C:mitochondrial inner membrane; IDA:MGI. DR InterPro; IPR006130; Asp/Orn_carbamoyltranf. DR InterPro; IPR006132; Asp/Orn_carbamoyltranf_P_bd. DR InterPro; IPR006131; Asp_carbamoyltransf_Asp/Orn_bd. DR InterPro; IPR002292; Orn_carbamltrans. DR Pfam; PF00185; OTCace; 1. DR Pfam; PF02729; OTCace_N; 1. DR PRINTS; PR00100; AOTCASE. DR PRINTS; PR00102; OTCASE. DR TIGRFAMs; TIGR00658; orni_carb_tr; 1. DR PROSITE; PS00097; CARBAMOYLTRANSFERASE; 1. PE 1: Evidence at protein level; KW Acetylation; Amino-acid biosynthesis; Arginine biosynthesis; KW Disease mutation; Mitochondrion; Transferase; Transit peptide; KW Urea cycle. FT TRANSIT 1 32 Mitochondrion. FT CHAIN 33 354 Ornithine carbamoyltransferase. FT /FTId=PRO_0000020335. FT REGION 90 94 Carbamoyl phosphate binding (By FT similarity). FT REGION 263 267 Ornithine binding (By similarity). FT REGION 302 305 Ornithine binding (By similarity). FT ACT_SITE 303 303 By similarity. FT BINDING 141 141 Carbamoyl phosphate (By similarity). FT BINDING 141 141 Ornithine (By similarity). FT BINDING 168 168 Carbamoyl phosphate (By similarity). FT BINDING 199 199 Ornithine (By similarity). FT BINDING 330 330 Carbamoyl phosphate (By similarity). FT BINDING 330 330 Ornithine (By similarity). FT MOD_RES 88 88 N6-acetyllysine. FT MOD_RES 231 231 N6-acetyllysine. FT MOD_RES 238 238 N6-acetyllysine. FT VARIANT 117 117 H -> N (in spf). FT CONFLICT 195 195 G -> R (in Ref. 2). SQ SEQUENCE 354 AA; 39765 MW; 33BBE5D1E88AA196 CRC64; MLSNLRILLN NAALRKGHTS VVRHFWCGKP VQSQVQLKGR DLLTLKNFTG EEIQYMLWLS ADLKFRIKQK GEYLPLLQGK SLGMIFEKRS TRTRLSTETG FALLGGHPSF LTTQDIHLGV NESLTDTARV LSSMTDAVLA RVYKQSDLDT LAKEASIPIV NGLSDLYHPI QILADYLTLQ EHYGSLKGLT LSWIGDGNNI LHSIMMSAAK FGMHLQAATP KGYEPDPNIV KLAEQYAKEN GTKLSMTNDP LEAARGGNVL ITDTWISMGQ EDEKKKRLQA FQGYQVTMKT AKVAASDWTF LHCLPRKPEE VDDEVFYSPR SLVFPEAENR KWTIMAVMVS LLTDYSPVLQ KPKF //