Glycine amidinotransferase, mitochondrial (EC 2.1.4.1) (L-arginine:glycine amidinotransferase) (Transamidinase)
1_MLRVR 6_ CLRGG 11_ SRGAE 16_ AVHYI 21_ GSRLG 26_ RTLTG 31_ WVQRT 36_ FQSTQ 41_ AATAS 46_ SRNSC 51_ AADDK 56_ ATEPL 61_ PKDCP 66_ VSSYN 71_ EWDPL 76_ EEVIV 81_ GRAEN 86_ ACVPP 91_ FTIEV 96_ KANTY 101_ EKYWP 106_ FYQKQ 111_ GGHYF 116_ PKDHL 121_ KKAVA 126_ EIEEM 131_ CNILK 136_ TEGVT 141_ VRRPD 146_ PIDWS 151_ LKYKT 156_ PDFES 161_ TGLYS 166_ AMPRD 171_ ILIVV 176_ GNEII 181_ EAPMA 186_ WRSRF 191_ FEYRA 196_ YRSII 201_ KDYFH 206_ RGAKW 211_ TTAPK 216_ PTMAD 221_ ELYNQ 226_ DYPIH 231_ SVEDR 236_ HKLAA 241_ QGKFV 246_ TTEFE 251_ PCFDA 256_ ADFIR 261_ AGRDI 266_ FAQRS 271_ QVTNY 276_ LGIEW 281_ MRRHL 286_ APDYR 291_ VHIIS 296_ FKDPN 301_ PMHID 306_ ATFNI 311_ IGPGI 316_ VLSNP 321_ DRPCH 326_ QIDLF 331_ KKAGW 336_ TIITP 341_ PTPII 346_ PDDHP 351_ LWMSS 356_ KWLSM 361_ NVLML 366_ DEKRV 371_ MVDAN 376_ EVPIQ 381_ KMFEK 386_ LGITT 391_ IKVNI 396_ RNANS 401_ LGGGF 406_ HCWTC 411_ DVRRR 416_GTLQS
1: Transamidinase that catalyzes the transfer of the amidino group of L-arginine onto the amino moiety of acceptor metabolites such as glycine, beta-alanine, gamma-aminobutyric acid (GABA) and taurine yielding the corresponding guanidine derivatives (PubMed:16820567, PubMed:27233232, PubMed:36543883, PubMed:3800397). Catalyzes the rate-limiting step of creatine biosynthesis, namely the transfer of the amidino group from L-arginine to glycine to generate guanidinoacetate, which is then methylated by GAMT to form creatine. Provides creatine as a source for ATP generation in tissues with high energy demands, in particular skeletal muscle, heart and brain (Probable) (PubMed:27233232, PubMed:36543883, PubMed:3800397, PubMed:9266688)