Ornithine transcarbamylase, mitochondrial (OTCase) (EC 2.1.3.3) (Ornithine carbamoyltransferase, mitochondrial)
1_MLFNL 6_ RILLN 11_ NAAFR 16_ NGHNF 21_ MVRNF 26_ RCGQP 31_ LQNKV 36_ QLKGR 41_ DLLTL 46_ KNFTG 51_ EEIKY 56_ MLWLS 61_ ADLKF 66_ RIKQK 71_ GEYLP 76_ LLQGK 81_ SLGMI 86_ FEKRS 91_ TRTRL 96_ STETG 101_ FALLG 106_ GHPCF 111_ LTTQD 116_ IHLGV 121_ NESLT 126_ DTARV 131_ LSSMA 136_ DAVLA 141_ RVYKQ 146_ SDLDT 151_ LAKEA 156_ SIPII 161_ NGLSD 166_ LYHPI 171_ QILAD 176_ YLTLQ 181_ EHYSS 186_ LKGLT 191_ LSWIG 196_ DGNNI 201_ LHSIM 206_ MSAAK 211_ FGMHL 216_ QAATP 221_ KGYEP 226_ DASVT 231_ KLAEQ 236_ YAKEN 241_ GTKLL 246_ LTNDP 251_ LEAAH 256_ GGNVL 261_ ITDTW 266_ ISMGQ 271_ EEEKK 276_ KRLQA 281_ FQGYQ 286_ VTMKT 291_ AKVAA 296_ SDWTF 301_ LHCLP 306_ RKPEE 311_ VDDEV 316_ FYSPR 321_ SLVFP 326_ EAENR 331_ KWTIM 336_ AVMVS 341_ LLTDY 346_SPQLQ
1: 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)