Immunofluorescence analysis of NIH3T3 cells using ASL antibody (green). Blue: DAPI fluorescent DNA dye. Red: Actin filaments have been labeled with Omnimabs® 594-Phalloidin.
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]
Alternative Names
argininosuccinase argininosuccinate lyase argininosuccinate lyase (EC 4.3.2.1) arginosuccinase ASAL
Immunofluorescence analysis of NIH3T3 cells using ASL antibody (green). Blue: DAPI fluorescent DNA dye. Red: Actin filaments have been labeled with Omnimabs® 594-Phalloidin.
Additional Information
Form
Liquid
Storage Instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C long term. Avoid freeze / thaw cycle.
Storage Buffer
Purified antibody in PBS with 0.05% PC300,40% Glycerol.