Adenylosuccinate lyase

Adenylosuccinate lyase
	Ribbon model of the tetramer, according to PDB 2J91
	Existing structural data : 2J91 , 2VD6 , 4FFX , 4FLC
Properties of human protein
Mass / length primary structure	483 amino acids
Isoforms	2
Identifier
Gene names	ADSL AMPS; ASASE; ASL
External IDs	OMIM : 103050 ; UniProt : P30566 ;
Enzyme classification
EC, category	4.3.2.2 , lyase
Response type	Splitting off of fumarate
Substrate	Adenylosuccinate / SAICAR
Products	Fumarate + AMP / AICAR
Occurrence
Parent taxon	Creature
	Orthologue

Adenylosuccinate lyase (ASase) (also: adenylosuccinase ) is an enzyme in all living things that catalyzes two chemical reactions in the metabolism of purines : the cleavage of fumarate from 5-aminoimidazole-4-N-succino-carboxamide-ribonucleotide (SAICAR) the de novo synthesis of inosine monophosphate (IMP); and of adenylosuccinate in the synthesis of adenosine monophosphate (AMP). Humans express ASase in all tissue types. Mutations in ADSL - gene are for Adenylosuccinase deficiency responsible for a rare hereditary disease.

Catalyzed reactions

⇔ +

Fumarate is split off from SAICAR, AICAR is created.

⇒ +

Fumarate is split off from adenyl succinate, producing AMP.

Spiegel EK, Colman RF, Patterson D: Adenylosuccinate lyase deficiency . In: Mol. Genet. Metab. . 89, No. 1-2, 2006, pp. 19-31. doi : 10.1016 / j.ymgme.2006.04.018 . PMID 16839792 .

Wikibooks: Purine Metabolism - Learning and Teaching Materials