ENTRY EC 2.3.1.37
NAME 5-Aminolevulinate synthase
CLASS Transferases
Acyltransferases
Acyltransferases
SYSNAME Succinyl-CoA:glycine C-succinyl-transferase(decarboxylating)
REACTION Succinyl-CoA + Glycine = 5-Aminolevulinate + CoA + CO2
SUBSTRATE Succinyl-CoA
Glycine
PRODUCT 5-Aminolevulinate
CoA
CO2
COFACTOR Pyridoxal phosphate
COMMENT A pyridoxal-phosphate protein. The enzyme in erythrocytes is
genetically distinct from that in other tissues.
PATHWAY PATH: MAP00260 Glycine, serine and threonine metabolism
DISEASE MIM: 125290 Aminolevulinate, delta-, synthase-1
MIM: 301300 Aminolevulinate, delta-, synthase-2; Anemia,
sideroblastic/hypochromic (3)
MOTIF PS: PS00599 T-[LIVMFYW]-[STAG]-K-[SAG]-[LIVMFYWR]-[SAG]-x(2)-[SAG]
GENES SCE: YDR232W(HEM1)
HSA: ALAS1(ALAS) ALAS2(ASB)
DBLINKS University of Geneva ENZYME DATA BANK: 2.3.1.37
WIT (What Is There) Metabolic Reconstruction: 2.3.1.37
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