The enzyme argininosuccinate lyase (EC 4.3.2.1, ASL, argininosuccinase; systematic name 2-(N ω-L-arginino)succinate arginine-lyase (fumarate-forming)) catalyzes the reversible breakdown of argininosuccinate: 2-(N ω-L-arginino)succinate = fumarate + L-arginine Located in liver cytosol, it is the fourth enzyme of the urea cycle and involved in the biosynthesis of arginine in all species and the production of urea in ureotelic species. Mutations resulting in low activity of the enzyme increase levels of urea in the body and result in various side effects.
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| - Argininosuccinate lyase (en)
- Argininosuccinat-Lyase (de)
- Argininosuccinate lyase (fr)
- 精氨基琥珀酸裂解酶 (zh)
- ASL (uk)
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| - Die Argininosuccinat-Lyase (ASL) ist das Enzym, das die Spaltung von Argininosuccinat in Arginin und Fumarat katalysiert. Diese Reaktion findet in allen Lebewesen als letzter Schritt der Arginin-Biosynthese statt, zählt aber auch in Wirbeltieren als Teilschritt des Harnstoffzyklus. Mutationen im ASL-Gen können ASL-Mangel verursachen, einem Harnstoffzyklusdefekt mit Ausscheidung von im Urin. ASL eignet sich aufgrund seiner spezifischen Expression als Laborwert für Lebererkrankungen. (de)
- L'argininosuccinate lyase (ASL) est une lyase qui catalyse la réaction : 2-(Nω-L-arginino)succinate fumarate + L-arginine. Cette enzyme cytosolique du foie intervient dans le cycle de l'urée et dans la biosynthèse de l'arginine chez tous les organismes, ainsi que dans la production d'urée chez les organismes (en). (fr)
- 精氨基琥珀酸裂解酶（英語：Argininosuccinate lyase，簡稱ASL），或稱精氨酸琥珀酸裂解酶是一種將精氨基琥珀酸分裂成精氨酸及延胡索酸的酶（EC4.3.2.1）。它負責尿素循環中的第4個步驟。 (zh)
- The enzyme argininosuccinate lyase (EC 4.3.2.1, ASL, argininosuccinase; systematic name 2-(N ω-L-arginino)succinate arginine-lyase (fumarate-forming)) catalyzes the reversible breakdown of argininosuccinate: 2-(N ω-L-arginino)succinate = fumarate + L-arginine Located in liver cytosol, it is the fourth enzyme of the urea cycle and involved in the biosynthesis of arginine in all species and the production of urea in ureotelic species. Mutations resulting in low activity of the enzyme increase levels of urea in the body and result in various side effects. (en)
- ASL (англ. Argininosuccinate lyase) – білок, який кодується однойменним геном, розташованим у людей на короткому плечі 7-ї хромосоми. Довжина поліпептидного ланцюга білка становить 464 амінокислот, а молекулярна маса — 51 658. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин (uk)
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| - Argininosuccinate lyase (en)
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| - Argininosuccinate lyase (en)
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| - Crystallographic structure of the human ASL monomer with labeled domains. (en)
- Crystal structure of duck argininosuccinate lyase with bound argininosuccinate. (en)
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