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| Cat.No | ACP22104 | Target Name | ASL |
|---|---|---|---|
| Target Synonyms | Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1 | Form | Lyophilized powder |
| Expression System | Custom Production. Please inquire and provide the desire expression system. | Expression Range | 2-464 |
| Protein Length | Full Length of Mature Protein | Purity | >85% (SDS-PAGE) |
| Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P04424 |
|---|
Uniprot Id
P04424
Target Species
Human
Target Name
ASL
Target Full Name
Argininosuccinate lyase
Target Involvement
Argininosuccinic aciduria (ARGINSA)
Target Protein Families
Lyase 1 family, Argininosuccinate lyase subfamily
Target Synonyms
Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1
Target Background
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.
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