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Literature summary extracted from

  • Bierau, J.; Pooters, I.N.; Visser, D.; Bakker, J.A.
    An HPLC-based assay of adenylosuccinate lyase in erythrocytes (2011), Nucleosides Nucleotides Nucleic Acids, 30, 908-917.
    View publication on PubMed

Inhibitors

EC Number Inhibitors Comment Organism Structure
4.3.2.2 CuSO4 complete inhibition at 0.05 mM Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.3.2.2 0.0091
-
 succinyladenosine monophosphate wild-type enzyme, pH 8.1, 37°C Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
4.3.2.2 1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole Homo sapiens
-
 5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
-
 ?
4.3.2.2 succinyladenosine monophosphate Homo sapiens
-
 AMP + fumarate
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
4.3.2.2 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.3.2.2 erythrocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.3.2.2 1-(5-phosphoribosyl)-4-(N-succinocarboxamide)-5-aminoimidazole
-
 Homo sapiens 5'-phosphoribosyl-5-amino-4-imidazolecarboxamide + fumarate
-
 ?
4.3.2.2 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 Homo sapiens ?
-
 ?
4.3.2.2 succinyladenosine monophosphate
-
 Homo sapiens AMP + fumarate
-
 ?

Synonyms

EC Number Synonyms Comment Organism
4.3.2.2 ADSL
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
4.3.2.2 malfunction ADSL enzyme deficiency is a disorder of purine metabolism with a broad clinical spectrum. The enzyme deficit causes a highly variable clinical presentation varying from a relatively mild presentation with slight motor retardation and severe muscle hypotonia to severe neonatal onset convulsions accompanied by severe mental retardation Homo sapiens
4.3.2.2 metabolism the enzyme is a bifunctional protein that is involved in both purine de novo synthesis and purine interconversion. It catalyzes the formation of phosphoribosylaminoimidazole carboxamide and succinate from phosphoribosylsuccinyl-aminoimidazole carboxamide in the biosynthesis of IMP from phosphoribosylpyrophosphate. Alternatively, the enzyme catalyzes the synthesis of AMP and succinate from succinyl-AMP in the interconversion of IMP into AMP Homo sapiens