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adenylosuccinate lyase (adenylosuccinase, ADSL, ASASE, AMPS, ASL)
Function:
- nucleotide biosynthesis; AMP biosynthesis; AMP from IMP: step 2/2
- nucleotide biosynthesis; IMP biosynthesis; 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide from N(2)-formyl-N1-(5-phospho-D-ribosyl)glycinamide: step 5/5
- context: purine biosynthesis
N(6)-(1,2-dicarboxyethyl)AMP fumarate + AMP
(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido)succinate
fumarate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide
Structure:
- belongs to the lyase 1 family, adenylosuccinate lyase subfamily
Alternative splicing: named isoforms=2
Expression:
- ubiquitously expressed
- both isoforms are produced by all tissues
Pathology:
- defects in ADSL are the cause of adenylosuccinase deficiency
Laboratory:
- adenylosuccinate lyase in erythrocytes
General
lyase
Properties
SIZE: entity length = 484 aa
MW = 55 kD
MOTIF: histidine residue {H86}
histidine residue {H159}
Database Correlations
OMIM 103050
UniProt P30566
Pfam PF00206
Entrez Gene 158
KEGG correlations
ENZYME 4.3.2.2
References
- UniProt :accession P30566
- ADSLdb; Adenylosuccinate lyase mutations database
http://www.icp.ucl.ac.be/adsldb/
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=ADSL