ASL Antibody

ASL is a member of the lyase 1 family. The 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 its gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency.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.

For short periods of storage (days) store at 4˚ C. For longer periods of storage, store ASL antibody at -20˚ C. As with any antibody avoid repeat freeze-thaw cycles.

ASL antibody can be used for detection of ASL by ELISA at 1:62500. ASL antibody can be used for detection of ASL by western blot at 5.0 μ g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.