2.3.1.37 2-hydroxybutanoyl-CoA + glycine - Mus musculus ? + CoA + CO2 - ? 407863 2.3.1.37 5-aminolevulinate-CoA weak cyclization activity Cereibacter sphaeroides 2-amino-3-hydroxycyclopent-2-en-1-one + CoASH - - 455103 2.3.1.37 acetyl-CoA + glycine low activity Cereibacter sphaeroides ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Gallus gallus ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Cavia porcellus ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Homo sapiens ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Rattus norvegicus ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Saccharomyces cerevisiae ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Oryctolagus cuniculus ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Cereibacter sphaeroides ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Spinacia oleracea ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Propionibacterium freudenreichii subsp. shermanii ? - ? 320843 2.3.1.37 acetyl-CoA + glycine at 2% the rate of the reaction with succinyl-CoA Rhodospirillum rubrum ? - ? 320843 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Gallus gallus 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Cavia porcellus 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Homo sapiens 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Rattus norvegicus 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Saccharomyces cerevisiae 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Oryctolagus cuniculus 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Cereibacter sphaeroides 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Spinacia oleracea 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Propionibacterium freudenreichii subsp. shermanii 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 alpha-glutamyl-CoA + glycine at 9% the rate of the reaction with succinyl-CoA Rhodospirillum rubrum 6-amino-5-oxohexanoate + CoA + CO2 - ? 320842 2.3.1.37 butanoyl-CoA + glycine - Mus musculus ? + CoA + CO2 - ? 408795 2.3.1.37 butyryl-CoA + glycine low activity Cereibacter sphaeroides ? - ? 320845 2.3.1.37 glutaryl-CoA + glycine - Mus musculus ? + CoA + CO2 - ? 409528 2.3.1.37 additional information enzyme deficiency causes X-linked sideroblastic anemia Homo sapiens ? - ? 89 2.3.1.37 additional information analysis of unstable enzyme-catalyzed reaction intermediates and conformational changes using physiological and non-physiological substrates and promiscuous T148A enzyme variant, overview. Formation of the quinonoid intermediate upon reacting glycine with the enzyme. Significantly, in the absence of the succinyl-CoA substrate, the external aldimine predominates over the glycine quinonoid intermediate. When instead of glycine, L-serine is reacted with the enzyme, a lag phase is observed in the progress curve for the L-serine external aldimine formation, indicating a hysteretic behavior in enzyme ALAS. Hysteresis is not detected in the T148A-catalyzed L-serine external aldimine formation. The rate of 5-aminolevulinate release is also controlled by a hysteretic kinetic mechanism. The active site residue Thr148 modulates the enzyme's strict amino acid substrate specificity, positioning of the glycine external aldimine in the active site, overview Mus musculus ? - ? 89 2.3.1.37 additional information the wild-type enzyme catalyzes the conversion of 5-aminolevulinate into the quinonoid intermediate at a rate 6.3fold slower than the formation of the same quinonoid intermediate from glycine and succinyl-CoA. The mutant N150F enzyme catalyzes the forward reaction at a 1.2fold faster rate than that of the reverse reaction, and the N150H variant reverses the rate values with a 1.7fold faster rate for the reverse reaction than that for the forward reaction. Steric constraints imposed by the active site of wild-type enzyme ALAS contribute toward the amino acid substrate specificity Mus musculus ? - ? 89 2.3.1.37 additional information no activity with L-alanine, L-serine or L-threonine Mus musculus ? - - 89 2.3.1.37 octanoyl-CoA + glycine - Mus musculus ? + CoA + CO2 - ? 409990 2.3.1.37 propionyl-CoA + glycine low activity Cereibacter sphaeroides ? - ? 320844 2.3.1.37 succinyl-CoA + glycine - Gallus gallus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Cavia porcellus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Paracoccus denitrificans 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Escherichia coli 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Saccharomyces cerevisiae 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Oryctolagus cuniculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Solanum tuberosum 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Rhodopseudomonas palustris 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Rhodobacter capsulatus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Plasmodium falciparum 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Acidipropionibacterium acidipropionici 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Rhodospirillum rubrum 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Serratia plymuthica 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Oncorhynchus mykiss 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Novispirillum itersonii 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine - Penaeus vannamei 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Homo sapiens 5-aminolevulinate + CoA + CO2 - r 320840 2.3.1.37 succinyl-CoA + glycine - Mus musculus 5-aminolevulinate + CoA + CO2 - r 320840 2.3.1.37 succinyl-CoA + glycine - Caulobacter vibrioides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Solanum tuberosum 5-aminolevulinate + CoA + CO2 common intermediate in the biosynthesis of chlorophyll and heme ? 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Gallus gallus 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Cavia porcellus 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Homo sapiens 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Saccharomyces cerevisiae 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Oryctolagus cuniculus 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Spinacia oleracea 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Rhodopseudomonas palustris 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Propionibacterium freudenreichii subsp. shermanii 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for glycine Rhodospirillum rubrum 5-aminolevulinate + CoA + CO2 - ir 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for succinyl-CoA Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for succinyl-CoA Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolute requirement for succinyl-CoA Rhodopseudomonas palustris 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine enzyme utilizes acetyl-CoA, propionyl-CoA and butyryl-CoA at much lower rates Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine glycine is formed by the reverse reaction under optimum conditions at 4-5% the rate of 5-aminolevulinate formed by the forward reaction under optimum conditions Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - r 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Gallus gallus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Paracoccus denitrificans 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Saccharomyces cerevisiae 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Euglena gracilis 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine absolutely specific for glycine Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Gallus gallus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Cavia porcellus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Saccharomyces cerevisiae 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Oryctolagus cuniculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Spinacia oleracea 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Propionibacterium freudenreichii subsp. shermanii 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Rhodospirillum rubrum 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine key enzyme in tetrapyrrole biosynthesis Novispirillum itersonii 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine cellular iron status plays a regulatory role Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine regulatory mechanisms in hepatic and erythroid cells Gallus gallus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine regulatory mechanisms in hepatic and erythroid cells Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine regulatory mechanisms in hepatic and erythroid cells Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine regulatory mechanisms in hepatic and erythroid cells Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine regulatory mechanisms in hepatic and erythroid cells Saccharomyces cerevisiae 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine regulatory mechanisms in hepatic and erythroid cells Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine first step in heme biosynthesis pathway Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Gallus gallus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Cavia porcellus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Rattus norvegicus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Saccharomyces cerevisiae 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Oryctolagus cuniculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Cereibacter sphaeroides 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Spinacia oleracea 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Propionibacterium freudenreichii subsp. shermanii 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine rate-limiting enzyme of heme biosynthesis Rhodospirillum rubrum 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine ALAS2 synthesizes heme specifically for haemoglobin Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine first and rate-limiting step of heme biosynthesis. Repression of 5-aminolevulinate synthase gene by the potent tumor promoter, TPA, involves multiple signal transduction pathways Homo sapiens 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine 5-aminolevulinate synthase operates under the stereoelectronic control predicted by Dunathan’s hypothesis Mus musculus 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine it is the CoA, rather than the succinyl moiety, that facilitates binding of succinyl-CoA to wild-type ALAS Mus musculus 5-aminolevulinate + CoA + CO2 - r 320840 2.3.1.37 succinyl-CoA + glycine - Caulobacter vibrioides CB15 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Cereibacter sphaeroides ATCC17023 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Cereibacter sphaeroides ATCC17029 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Rhodopseudomonas palustris KUGB306 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Rhodobacter capsulatus ATCC BAA-309 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Cereibacter sphaeroides Y 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + glycine - Cereibacter sphaeroides ATCC17025 5-aminolevulinate + CoA + CO2 - ? 320840 2.3.1.37 succinyl-CoA + L-serine the reaction with L-serine follows a biphasic kinetic process Mus musculus ? - ? 435839 2.3.1.37 succinyl-CoA + O-methylglycine - Mus musculus ? - ? 435840 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Gallus gallus 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Cavia porcellus 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Homo sapiens 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Rattus norvegicus 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Saccharomyces cerevisiae 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Oryctolagus cuniculus 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Cereibacter sphaeroides 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Spinacia oleracea 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Propionibacterium freudenreichii subsp. shermanii 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841 2.3.1.37 succinyl-CoA-monomethyl ester + glycine at 80% the rate of the reaction with succinyl-CoA Rhodospirillum rubrum 5-aminolevulinic acid methyl ester + CoA + CO2 - ? 320841