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aminolevulinic acid [ALA] synthetase
1st & rate limiting enzyme in heme biosynthetic pathway; catalyzes condensation of glycine with succinyl-CoA to form delta-aminolevulinic acid
Interactions
molecular events
Related
heme synthesis
Specific
ALA synthetase-1 (nonspecific 5-aminolevulinate synthetase mitochondrial, ALAS-H, ALAS1, ALAS3, ALASH, OK/SW-cl121)
ALA synthetase-2 (ALAS2, erythroid-specific 5-aminolevulinate synthetase, erythrocytic aminolevulinate synthetase)
General
acyltransferase
allosteric enzyme
oligomerizing protein
Properties
CONFIGURATION: dimer
SIZE: MW = 130 kD
COMPARTMENT: mitochondrial matrix
MOTIF: cofactor-binding site
COFACTOR-BOUND: pyridoxal phosphate
allosteric site
MISC-INFO: Ki [heme] 0.5 UM
1/2life 60 MIN
Database Correlations
ENZYME 2.3.1.37
References
Textbook of Biochemistry with Clinical Correlations,
3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 1012