4.3.2.2: adenylosuccinate lyase
This is an abbreviated version!
For detailed information about adenylosuccinate lyase, go to the full flat file.
Word Map on EC 4.3.2.2
-
4.3.2.2
-
purine
-
saicar
-
succinyladenosine
-
autistic
-
carboxamide
-
psychomotor
-
imp
-
succinylaminoimidazole
-
amylosucrase
-
ribotide
-
geothermalis
-
aminoimidazole
-
drug development
-
medicine
- 4.3.2.2
- purine
-
saicar
-
succinyladenosine
-
autistic
- carboxamide
-
psychomotor
- imp
-
succinylaminoimidazole
- amylosucrase
- ribotide
- geothermalis
-
aminoimidazole
- drug development
- medicine
Reaction
Synonyms
adenylosuccinase, adenylosuccinate lyase, ADL, ADSL, AMPS lyase, ASASE, ASL, Glutamyl-tRNA synthetase regulatory factor, lyase, adenylosuccinate, PurB, succino AMP-lyase, succino-AMP lyase
ECTree
4.3.2.2 adenylosuccinate lyaseAdvanced search results
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results (Substrates Products
Substrates Products on EC 4.3.2.2 - adenylosuccinate lyase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole
5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
2',3'-dideoxyadenylosuccinate
dideoxyadenosine 5'-monophosphate + ?
-
at 1.44% of the activity with the natural substrate
-
?
4-(N-succino)-5-aminoimidazole-4-carboxamide ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
adenylosuccinate 2',3'-acyclic dialcohol
adenylic 2',3'-acyclic dialcohol + fumarate
-
-
-
-
?
phosphoribosylsuccinyl-aminoimidazole carboxamide
phosphoribosylaminoimidazole carboxamide + fumarate
-
-
-
-
?
succino-4-aminopyrazolo[3,4-d]pyrimidine ribose 5'-phosphate
4-aminopyrazolo[3,4-d]pyrimidine ribose 5'-phosphate + ?
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
the enzyme is involved in both de novo and salvage pathways of purine biosynthesis
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
?
(S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
-
-
-
?
1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole
5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
-
-
-
-
?
1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole
5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
-
-
-
?
1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole
5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
-
-
-
?
1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole
5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
-
i.e. 4-(N-succino)-5-aminoimidazole-4-carboxamide ribonucleotide ?
-
-
?
4-(N-succino)-5-aminoimidazole-4-carboxamide ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
reaction in the biosynthesis of AMP
-
-
?
4-(N-succino)-5-aminoimidazole-4-carboxamide ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
enzyme catalyzes two nonsequential reactions in purine biosynthesis
-
-
?
4-(N-succino)-5-aminoimidazole-4-carboxamide ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
reaction in the biosynthesis of AMP
-
-
?
4-(N-succino)-5-aminoimidazole-4-carboxamide ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
reaction in the biosynthesis of AMP
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
-
?
5-aminoimidazole-(N-succinylocarboxamide) ribotide
5-aminoimidazole-4-carboxamide ribotide + fumarate
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
i.e. SAICAR
i.e. AICAR
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
i.e. SAICAR
i.e. AICAR
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
ZMP accumulation in the brain of Lesch-Nyhan disease patients induces inhibition of mitochondrial oxidative phosphorylation and adenylosuccinate lyase activity, followed by dowregulation of ATP levels and multifocal cell death in the cerebellum. ZMP is an inhibitor of the bifunctional enzyme adenylosuccinate lyase, a deficiency of this enzyme causes psychomotor and mental retardation in humans, overview, i.e. AICAR or ZMP
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
i.e. SAICAR
i.e. AICAR
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
i.e. SAICAR
i.e. AICAR
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
the 8th step in the purine nucleotide de novo pathway
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
first step common to both de novo and the salvage pathways in purine biosynthesis
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
i.e. SAICAR, SAICAR synthesis in vitro
i.e. AICAR
-
r
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
-
?
5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate
-
-
-
-
?
adenylosuccinate
AMP + fumarate
-
-
-
-
?
arabinosyl-succino-AMP
arabinosyl-AMP + ?
-
i.e. beta-D-arabinosyladenylosuccinate
beta-D-arabinosyl-AMP
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
reaction in the biosynthesis of AMP
-
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
the enzyme is involved in both de novo and salvage pathways of purine biosynthesis
-
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
the enzyme follows a Uni-Bi ordered kinetic mechanism, in which release of fumarate is followed by AMP to yield free enzyme
-
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
terminal step in AMP biosynthesis
-
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
reaction in the biosynthesis of AMP
-
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
reaction in the biosynthesis of AMP
-
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
i.e. adenylosuccinate
-
?
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
i.e. adenylosuccinate
-
r
N6-(1,2-dicarboxyethyl)AMP
fumarate + AMP
-
enzyme catalyzes the last step in the pathway of AMP biosynthesis
-
-
?
succinyladenosine monophosphate
AMP + fumarate
-
-
-
-
?
succinyladenosine monophosphate
AMP + fumarate
-
conversion of adenylosuccinate to adenylate in the purine nucleotide cycle
-
-
?
succinyladenosine monophosphate
AMP + fumarate
second step common to both de novo and the salvage pathways in purine biosynthesis
-
-
?
additional information
?
-
-
adenylosuccinate lyase catalyzes two reactions in the biosynthesis of purine nucleotides
-
-
?
additional information
?
-
-
residues Ser262 and Ser263 in a flexible loop of the enzyme are essential for catalysis
-
-
?
additional information
?
-
adenylosuccinate lyase catalyzes two reactions in the biosynthesis of purine nucleotides
-
-
?
additional information
?
-
-
adenylosuccinate lyase catalyzes two reactions in the biosynthesis of purine nucleotides
-
-
?
additional information
?
-
adenylosuccinate lyase deficiency is a rare autosomal disorder of de novo purine synthesis, which results in the accumulation of succinylpurines in body fluids. Patients with adenylosuccinate lyase deficiency show a variable combination of mental retardation, epilepsy and autistic features, overview
-
-
?
additional information
?
-
-
adenylosuccinate lyase deficiency is a rare autosomal disorder of de novo purine synthesis, which results in the accumulation of succinylpurines in body fluids. Patients with adenylosuccinate lyase deficiency show a variable combination of mental retardation, epilepsy and autistic features, overview
-
-
?
additional information
?
-
-
adenylosuccinate lyase deficiency is an autosomal recessive disorder of the purine de novo synthesis pathway and purine nucleotide cycleby severe neurological involvement including hypotonia, seizures, developmental delay and autistic features. Epilepsy in ADSL deficiency is frequent and occurs in approximately two-thirds of patients, beginning either early in the neonatal period or after the first year of life
-
-
?
additional information
?
-
-
ZMP is an activator of the AMP-activated protein kinase, AMPK, and is toxic in human B lymphocytes and neuroblastoma cell lines, physiological functions of ZMP, overview
-
-
?
additional information
?
-
residue Ser289 is essential for catalysis
-
-
?
additional information
?
-
-
development of a rapid and simple HPLC-based assay method to quantitatively measure ADSL activity with succinyladenosine monophosphate in erythrocytes using isocratic ionpairing reversed-phase HPLC with UV-detection, overview
-
-
?
additional information
?
-
interaction between the enzyme and phosphoribosylsuccinyl-aminoimidazole carboxamide, isothermal titration calorimetry, overview. Modeling of AMPand phosphoribosylaminoimidazole carboxamide, and fumarate binding in the active site of wild-type and R303C mutant enzymes
-
-
?
additional information
?
-
-
interaction between the enzyme and phosphoribosylsuccinyl-aminoimidazole carboxamide, isothermal titration calorimetry, overview. Modeling of AMPand phosphoribosylaminoimidazole carboxamide, and fumarate binding in the active site of wild-type and R303C mutant enzymes
-
-
?
additional information
?
-
-
the activity of the Mycobacterium tuberculosis enzyme is particularly low
-
-
?
additional information
?
-
adenylosuccinate lyase is the only enzyme in the purine biosynthetic pathway that catalyzes two distinct, but chemically similar reactions
-
-
?
additional information
?
-
-
adenylosuccinate lyase is the only enzyme in the purine biosynthetic pathway that catalyzes two distinct, but chemically similar reactions
-
-
?
additional information
?
-
-
the low activity of the enzyme in liver of severely starved rats is inconsistent with the proposal that the purine nucleotide cycle plays a major role in ammonia production for urea synthesis at least under these conditions
-
-
?
additional information
?
-
-
roles of adenylosuccinate lyase and of AMP deaminase in the anti-HIV activity of 2',3'-dideoxyadenosine and 2',3'-dideoxyinosine
-
-
?
