人 Liver Arginase 抗体对
01AB04224
Arginase-1 (ARG1) is a crucial enzyme in the urea cycle, primarily responsible for converting L-arginine into L-ornithine and urea, thus facilitating the detoxification of ammonia in mammals. This enzyme is a member of the ureohydrolase family and operates as a trimer, requiring manganese ions for its catalytic activity. It plays a significant role in nitrogen metabolism by removing excess nitrogen from the body, which is particularly important in liver cells where it is predominantly expressed. The enzyme's activity is tightly regulated, as alterations can impact various physiological processes, including immune responses and cellular signaling pathways. Mutations or deficiencies in ARG1 can lead to arginase deficiency, a rare metabolic disorder characterized by hyperargininemia, which results in elevated levels of arginine and ammonia in the blood. This condition can cause severe neurological symptoms, including developmental delays and spasticity due to ammonia's neurotoxic effects.
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技术参数
- 靶点基因名
- ARG1
- 蛋白别名
- arginase 1; Liver Arginase
- UniProt
- P05089
- 物种
- Human
产品详情
- 靶点名称
- Liver Arginase
- 蛋白名称
- arginase 1
- 蛋白别名
- Arginase-1; Liver-type arginase; Type I arginase
- 靶点基因名
- ARG1
- UniProt
- P05089
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Arginase-1 (ARG1) is a crucial enzyme in the urea cycle, primarily responsible for converting L-arginine into L-ornithine and urea, thus facilitating the detoxification of ammonia in mammals. This enzyme is a member of the ureohydrolase family and operates as a trimer, requiring manganese ions for its catalytic activity. It plays a significant role in nitrogen metabolism by removing excess nitrogen from the body, which is particularly important in liver cells where it is predominantly expressed. The enzyme's activity is tightly regulated, as alterations can impact various physiological processes, including immune responses and cellular signaling pathways. Mutations or deficiencies in ARG1 can lead to arginase deficiency, a rare metabolic disorder characterized by hyperargininemia, which results in elevated levels of arginine and ammonia in the blood. This condition can cause severe neurological symptoms, including developmental delays and spasticity due to ammonia's neurotoxic effects.