Entrez Summary: This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008].
UniProt Summary: N/A
Pfam DomainsGO Terms
Pfam Domains
Lyase 1
GO Terms
argininosuccinate lyase activity
arginine biosynthetic process via ornithine
arginine biosynthetic process
ornithine metabolic process
urea cycle
urea metabolic process
nitrogen cycle metabolic process
arginine metabolic process
glutamine family amino acid biosynthetic process
alpha-amino acid biosynthetic process
glutamine family amino acid metabolic process
cellular amino acid biosynthetic process
alpha-amino acid metabolic process
carboxylic acid biosynthetic process
organic acid biosynthetic process
cellular amino acid metabolic process
amide biosynthetic process
small molecule biosynthetic process
cellular amide metabolic process
carboxylic acid metabolic process
oxoacid metabolic process
organic acid metabolic process
identical protein binding
organonitrogen compound biosynthetic process
cellular nitrogen compound biosynthetic process
small molecule metabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)
Compound Hit
No hits were found.
Most Correlated Genes in Chemogenomics
No correlation found to any other genes in chemogenomics.
Tissues where Essential in the Avana Dataset (DepMap 20Q1)
Global Fraction of Cell Lines Where Essential: 0/739