Argininosuccinate lyase
| Argininosuccinate lyase | |||||||||
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Crystal structure of duck argininosuccinate lyase with bound argininosuccinate. | |||||||||
| Identifiers | |||||||||
| EC no. | 4.3.2.1 | ||||||||
| CAS no. | 9027-34-3 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
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| Argininosuccinate lyase | |||||||
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Crystallographic structure of the human ASL monomer with labeled domains. | |||||||
| Identifiers | |||||||
| Symbol | ASL | ||||||
| NCBI gene | 435 | ||||||
| HGNC | 746 | ||||||
| OMIM | 608310 | ||||||
| RefSeq | NM_000048 | ||||||
| UniProt | P04424 | ||||||
| Other data | |||||||
| EC number | 4.3.2.1 | ||||||
| Locus | Chr. 7 pter-q22 | ||||||
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The enzyme argininosuccinate lyase (EC 4.3.2.1, ASL, argininosuccinase; systematic name 2-(N ω-L-arginino)succinate arginine-lyase (fumarate-forming)) catalyzes the reversible breakdown of argininosuccinate:
- 2-(N ω-L-arginino)succinate = fumarate + L-arginine
Located in liver cytosol, it is the fourth enzyme of the urea cycle and involved in the biosynthesis of arginine in all species and the production of urea in ureotelic species. Mutations resulting in low activity of the enzyme increase levels of urea in the body and result in various side effects.
The ASL gene is located on chromosome 7 between the centromere (junction of the long and short arm) and the long (q) arm at position 11.2, from base pair 64,984,963 to base pair 65,002,090.
ASL is related to intragenic complementation.