N-acetylglutamate synthase, mitochondrial (EC 2.3.1.1) (Amino-acid acetyltransferase) [Cleaved into: N-acetylglutamate synthase long form; N-acetylglutamate synthase short form; N-acetylglutamate synthase conserved domain form]
1_MATAL 6_ MAVVL 11_ RAAAV 16_ APRLR 21_ GRGGT 26_ GGARR 31_ LSCGA 36_ RRRAA 41_ RGTSP 46_ GRRLS 51_ TAWSQ 56_ PQPPP 61_ EEYAG 66_ ADDVS 71_ QSPVA 76_ EEPSW 81_ VPSPR 86_ PPVPH 91_ ESPEP 96_ PSGRS 101_ LVQRD 106_ IQAFL 111_ NQCGA 116_ SPGEA 121_ RHWLT 126_ QFQTC 131_ HHSAD 136_ KPFAV 141_ IEVDE 146_ EVLKC 151_ QQGVS 156_ SLAFA 161_ LAFLQ 166_ RMDMK 171_ PLVVL 176_ GLPAP 181_ TAPSG 186_ CLSFW 191_ EAKAQ 196_ LAKSC 201_ KVLVD 206_ ALRHN 211_ AAAAV 216_ PFFGG 221_ GSVLR 226_ AAEPA 231_ PHASY 236_ GGIVS 241_ VETDL 246_ LQWCL 251_ ESGSI 256_ PILCP 261_ IGETA 266_ ARRSV 271_ LLDSL 276_ EVTAS 281_ LAKAL 286_ RPTKI 291_ IFLNN 296_ TGGLR 301_ DSSHK 306_ VLSNV 311_ NLPAD 316_ LDLVC 321_ NAEWV 326_ STKER 331_ QQMRL 336_ IVDVL 341_ SRLPH 346_ HSSAV 351_ ITAAS 356_ TLLTE 361_ LFSNK 366_ GSGTL 371_ FKNAE 376_ RMLRV 381_ RSLDK 386_ LDQGR 391_ LVDLV 396_ NASFG 401_ KKLRD 406_ DYLAS 411_ LRPRL 416_ HSIYV 421_ SEGYN 426_ AAAIL 431_ TMEPV 436_ LGGTP 441_ YLDKF 446_ VVSSS 451_ RQGQG 456_ SGQML 461_ WECLR 466_ RDLQT 471_ LFWRS 476_ RVTNP 481_ INPWY 486_ FKHSD 491_ GSFSN 496_ KQWIF 501_ FWFGL 506_ ADIRD 511_ SYELV 516_ NHAKG 521_ LPDSF 526_HKPAS
1: Plays a role in the regulation of ureagenesis by producing the essential cofactor N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPS1) activity