argininosuccinate <=> fumarate + arginine

Stable Identifier
R-HSA-70573
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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Cytosolic argininosuccinate lyase (ASL) catalyzes the reversible reaction of argininosuccinate to form fumarate and arginine. The enzyme is a homotetramer (Turner et al. 1997). The function of the human enzyme in vivo is inferred from the defective argininosuccinate lyase enzyme activity observed in patients with mutant forms of the ASL gene (e.g., Walker et al. 1990).
Literature References
PubMed ID Title Journal Year
9256435 Human argininosuccinate lyase: a structural basis for intragenic complementation

Howell, PL, Turner, MA, McInnes, RR, Simpson, A

Proc Natl Acad Sci U S A 1997
2263616 Molecular analysis of human argininosuccinate lyase: mutant characterization and alternative splicing of the coding region

McCloskey, DA, McInnes, RR, Simard, LR, Walker, DC

Proc Natl Acad Sci U S A 1990
Participants
Participates
Catalyst Activity

argininosuccinate lyase activity of ASL tetramer [cytosol]

Orthologous Events
Cross References
Rhea
Authored
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