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.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:100ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by affinity chromatography.
Form:Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Specificity:Recognizes human ASL.
Isotype:IgG
Calc Applications Abbrev:WB
Calc Crossreactivity:Hu
Immunogen:Synthetic peptide corresponding to the N-terminal region of human ASL