Adenylosuccinate lyase
Adenylosuccinate lyase | ||
---|---|---|
Ribbon model of the tetramer, according to PDB 2J91 | ||
Properties of human protein | ||
Mass / length primary structure | 483 amino acids | |
Isoforms | 2 | |
Identifier | ||
Gene names | ADSL AMPS; ASASE; ASL | |
External IDs | ||
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 | ||
human | House mouse | |
Entrez | 158 | 11564 |
Ensemble | ENSG00000239900 | ENSMUSG00000022407 |
UniProt | P30566 | P54822 |
Refseq (mRNA) | NM_000026 | NM_009634 |
Refseq (protein) | NP_000017 | NP_033764 |
Gene locus | Chr 22: 40.35 - 40.37 Mb | Chr 15: 80.95 - 80.97 Mb |
PubMed search | 158 |
11564
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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.
literature
- 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 .
Individual evidence
Web links
Wikibooks: Purine Metabolism - Learning and Teaching Materials
- D'Eustachio / reactome: 5'-phosphoribosyl-5-aminoimidazole-4-N-succinocarboxamide (SAICAR) ⇔ 5'-phosphoribosyl-5-aminoimidazole-4-carboxamide (AICAR) + fumarate
- D'Eustachio / reactome: adenylosuccinate ⇒ adenosine 5'-monophosphate + fumarate
- OrphaNet: Adenylosuccinate Lyase Deficiency