抗人 ASL 抗体对
01AB01019
Argininosuccinate lyase (ASL) is a cytosolic enzyme that catalyzes the reversible cleavage of L-argininosuccinate into fumarate and L-arginine, a critical step in the urea cycle responsible for detoxifying ammonia in the liver. This reaction not only facilitates the removal of excess nitrogen by producing urea for excretion but also generates arginine, an amino acid essential for protein synthesis and nitric oxide production, which plays a key role in vascular regulation. The enzyme’s mechanism involves an E1cb elimination reaction, with specific amino acids such as histidine, threonine, lysine, and serine contributing to proton abstraction and stabilization of intermediates during catalysis. Deficiency of ASL due to mutations in the ASL gene leads to argininosuccinic aciduria, a rare inherited disorder characterized by hyperammonemia, developmental delay, and liver dysfunction, underscoring the enzyme’s importance in nitrogen metabolism and overall health.
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技术参数
- 靶点基因名
- ASL
- 蛋白别名
- argininosuccinate lyase; ASL
- UniProt
- P04424
- 物种
- Human
产品详情
- 靶点名称
- ASL
- 蛋白名称
- argininosuccinate lyase
- 蛋白别名
- Argininosuccinate lyase; ASAL; Arginosuccinase
- 靶点基因名
- ASL
- UniProt
- P04424
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Argininosuccinate lyase (ASL) is a cytosolic enzyme that catalyzes the reversible cleavage of L-argininosuccinate into fumarate and L-arginine, a critical step in the urea cycle responsible for detoxifying ammonia in the liver. This reaction not only facilitates the removal of excess nitrogen by producing urea for excretion but also generates arginine, an amino acid essential for protein synthesis and nitric oxide production, which plays a key role in vascular regulation. The enzyme’s mechanism involves an E1cb elimination reaction, with specific amino acids such as histidine, threonine, lysine, and serine contributing to proton abstraction and stabilization of intermediates during catalysis. Deficiency of ASL due to mutations in the ASL gene leads to argininosuccinic aciduria, a rare inherited disorder characterized by hyperammonemia, developmental delay, and liver dysfunction, underscoring the enzyme’s importance in nitrogen metabolism and overall health.