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Immunogen sequence: QTKILGSDR VKLMESLVVG DIAELRPNQG TLSLFTNEAG GILDDLIVTN TSEGHLYVVS NAGCWEKDLA LMQDKVRELQ NQGRDVGLEV LDNALLALQG PTAAQVLQAG VADDLRKLPF MTSAVMEVFG VSGCRVTRCG YTGEDGVEIS VPVAGAVHLA TAILKNPEVK LAGLAARDSL RLEAGLCLYG NDIDEHTTPV EGSLSWTLGK RRRAAMDFPG AKVIVPQLKG RVQRRRVGLM CEGAPMRAHS PILNMEGTKI GTVTSGCPSP SLKKNVAMGY VPCEYSRPGT MLLVELPSGP CF (83-383 aa encoded by BC007546)
The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). Glycine encephalopathy (GCE) may be due to a defect in any one of these enzymes.
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Protein Aliases: Aminomethyltransferase, mitochondrial; GCVT; Glycine cleavage system T protein
Gene Aliases: AMT; EG434437; GCE; GCST; GCVT; NKH
UniProt ID: (Human) P48728, (Mouse) Q8CFA2
Entrez Gene ID: (Human) 275, (Mouse) 434437
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