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Argininosuccinate Lyase

An enzyme of the urea cycle which splits argininosuccinate to fumarate plus arginine. Its absence leads to the metabolic disease ARGININOSUCCINIC ACIDURIA in man. EC 4.3.2.1.
Also Known As:
Lyase, Argininosuccinate; Argininosuccinase; N-(L-Argininosuccinate) arginine-lyase
Networked: 71 relevant articles (1 outcomes, 5 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1. Häberle, Johannes: 6 articles (11/2015 - 10/2002)
2. Nuoffer, Jean-Marc: 4 articles (09/2015 - 01/2012)
3. Eggimann, Sandra: 3 articles (09/2015 - 01/2012)
4. Hu, Liyan: 3 articles (09/2015 - 01/2012)
5. Rüfenacht, Véronique: 3 articles (09/2015 - 11/2013)
6. Pandey, Amit V: 3 articles (09/2015 - 11/2013)
7. Lai, Ming-Derg: 2 articles (12/2015 - 11/2013)
8. Cho, Chien-Yu: 2 articles (12/2015 - 11/2013)
9. Hsu, Hui-Ping: 2 articles (12/2015 - 11/2013)
10. Chen, Wei-Ching: 2 articles (12/2015 - 11/2013)

Related Diseases

1. Citrullinemia
04/01/1999 - "We also used MS/MS analysis of urine or blood either for confirmation of initial positive results or for follow-up of treatment, such as in glutaric acidemia, citrullinemia, argininosuccinase deficiency, and biopterin-dependent phenylketonuria."
03/01/2010 - "Argininosuccinate synthetase deficiency was the most frequent disease (17/37, 46%), followed by ornithine transcarbamylase (10/37, 27%), arginase (7/37, 19%), and argininosuccinate lyase (3/37, 8%) deficiencies. "
04/01/1976 - "Increased urinary levels of N-carbamoyl-beta-alanine, and also, on occasions, of N-carbamoylaspartate, were observed in patients with ornithine carbamoyl-transferase (EC 2.1.3.3) deficiency, argininosuccinate synthetase (EC 6.3.4.5) deficiency and argininosuccinate lyase (EC 4.3.2.1)deficiency, but not in a patient with carbamoylphosphate synthase deficiency. "
10/01/2013 - "This encompasses 5 major disorders, corresponding with deficiency of each step in the urea cycle, namely ornithine transcarbamoylase (OTC) deficiency, argininosuccinate lyase (ASL) deficiency, carbamoyl phosphate synthetase (CPS) deficiency, citrullinemia and argininemia. "
12/05/2002 - "In this review, the differential diagnosis for hyperammonemia are described; deficiencies of ornithine carbamoyl transferase, argininosuccinate synthase (citrullinemia), argininosuccinate lyase and arginase, lysinuric protein intolerance, hyperammonemia-hyperornithinemia-homocitrullinemia syndrome, and citrullinemia type II. The diagnosis of IEM of purine and pyrimidine such as deficiencies of hypoxanthine-guanine phosphoribosyl transferase, adenine phosphoribosyl transferase, dihydropyrimidine dehydrogenase, dihydropyrimidinase and beta-ureidopropionase are described. "
2. Phenylketonurias (Phenylketonuria)
3. Liver Diseases (Liver Disease)
4. Neoplasms (Cancer)
5. Anoxia (Hypoxia)

Related Drugs and Biologics

1. Biopterin
2. Argininosuccinate Synthase (Argininosuccinate Synthetase)
3. Urea (Carbamide)
4. Ornithine Carbamoyltransferase (Ornithine Transcarbamylase)
5. Arginase
6. Enzymes
7. Nitrogen
8. Protein Isoforms (Isoforms)
9. Superoxide Dismutase
10. Arginine (L-Arginine)

Related Therapies and Procedures

1. Protein-Restricted Diet (Diet, Protein Restricted)