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.1) Trevisson, E., (2007) Hum. Mutat. 28 (7), 694-702.
Immunogen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human ASL.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ELISAWB
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 0.25 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Antibody used in WB on Transfected 293T at 0.2-1 ug/ml.
Positive Control
1) Tranfected 293T Cell Lysate
Application Notes
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 0.25 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Additional Information
Form
Liquid
Storage Instructions
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.
Storage Buffer
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL.