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argininosuccinate lyase (argininosuccinase, ASAL, ASL)
Function:
- amino-acid biosynthesis; L-arginine biosynthesis
- L-arginine from L-ornithine & carbamoyl phosphate: step 3/3
- nitrogen metabolism; urea cycle
- L-arginine & fumarate from N(omega)-L-argininosuccinate: step 1/1
2-(N(omega)-L-argininosuccinate fumarate + L-arginine
Structure:
- homotetramer
- belongs to the lyase 1 family, argininosuccinate lyase subfamily
Pathology:
- defects in ASL are the cause of arginosuccinicaciduria
Laboratory:
- argininosuccinate lyase in fibroblasts
- argininosuccinate lyase in chorionic villus
- argininosuccinate lyase in liver
- argininosuccinate lyase in liver from fetus
- argininosuccinate lyase in serum
Interactions
molecular events
Related
argininosuccinate lyase deficiency; argininosuccinic aciduria; argininosuccinic acidemia
urea cycle
General
lyase
urea cycle enzyme
Properties
COMPARTMENT: cytoplasm
CELL: hepatocyte
MOTIF: active site
Database Correlations
OMIM 207900
UniProt P04424
Pfam PF00206
Entrez Gene 435
Kegg hsa:435
ENZYME 4.3.2.1
References
- OMIM :accession 207900
- UniProt :accession P04424
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=AS