Protein: Glycine amidinotransferase (A0A6Q8PHC6)
Organism: Homo sapiens (Human)
Function: 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. 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
Subcellular location: Mitochondrion inner membrane
InterPro domains:
- IPR033195: Glycine/inosamine-phosphate amidinotransferase (family) [3-402]
Sequence:
MRSLGRTLTGWVQRTFQSTQAATASSRNSCAADDKATEPLPKDCPVSSYNEWDPLEEVIVGRAENACVPPFTIEVKANTYEKYWPFYQKQGGHYFPKDHLKKAVAEIEEMCNILKTEGVTVRRPDPIDWSLKYKTPDFESTGLYSAMPRDILIVVGNEIIEAPMAWRSRFFEYRAYRSIIKDYFHRGAKWTTAPKPTMADELYNQDYPIHSVEDRHKLAAQGKFVTTEFEPCFDAADFIRAGRDIFAQRSQVTNYLGIEWMRRHLAPDYRVHIISFKDPNPMHIDATFNIIGPGIVLSNPDRPCHQIDLFKKAGWTIITPPTPIIPDDHPLWMSSKWLSMNVLMLDEKRVMVDANEVPIQKMFEKLGITTIKVNIRNANSLGGGFHCWTCDVRRRGTLQSYLD
Predicted GO terms:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016740 (transferase activity), GO:0005515 (protein binding), GO:0016741 (transferase activity, transferring one-carbon groups)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0050896 (response to stimulus), GO:0009987 (cellular process), GO:0032501 (multicellular organismal process), GO:0009058 (biosynthetic process), GO:0044237 (cellular metabolic process), GO:0043500 (muscle adaptation), GO:0044281 (small molecule metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0071704 (organic substance metabolic process), GO:0003008 (system process), GO:0007610 (behavior), GO:0003012 (muscle system process), GO:0006575 (cellular modified amino acid metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:1901576 (organic substance biosynthetic process), GO:0044283 (small molecule biosynthetic process), GO:0007611 (learning or memory), GO:0006082 (organic acid metabolic process), GO:0044249 (cellular biosynthetic process), GO:0050877 (nervous system process), GO:0014889 (muscle atrophy), GO:1901566 (organonitrogen compound biosynthetic process), GO:0050890 (cognition), GO:0042398 (cellular modified amino acid biosynthetic process), GO:0043436 (oxoacid metabolic process), GO:0016053 (organic acid biosynthetic process), GO:0019752 (carboxylic acid metabolic process), GO:0046394 (carboxylic acid biosynthetic process), GO:0072330 (monocarboxylic acid biosynthetic process), GO:0032787 (monocarboxylic acid metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)