抗人 Sulfite oxidase 抗体对
01AB00695
Sulfite oxidase (SUOX) is a homodimeric enzyme localized to the intermembrane space of mitochondria, crucial for the oxidative degradation of sulfur-containing amino acids. Each subunit includes a heme domain and a molybdopterin-binding domain, catalyzing the oxidation of sulfite to sulfate. The enzyme itself is a single polypeptide chain with a molecular mass of approximately 53.9 kDa, as seen in recombinant forms produced in E. coli. The SUOX gene provides instructions for making this enzyme, and mutations in this gene can lead to isolated sulfite oxidase deficiency (ISOD), a severe disorder causing brain damage and often fatal in early life due to the accumulation of toxic sulfite compounds in the nervous system.
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技术参数
- 靶点基因名
- SUOX
- 蛋白别名
- sulfite oxidase; Sulfite oxidase
- UniProt
- P51687
- 物种
- Human
产品详情
- 靶点名称
- Sulfite oxidase
- 蛋白名称
- sulfite oxidase
- 蛋白别名
- Sulfite oxidase; mitochondrial
- 靶点基因名
- SUOX
- UniProt
- P51687
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Sulfite oxidase (SUOX) is a homodimeric enzyme localized to the intermembrane space of mitochondria, crucial for the oxidative degradation of sulfur-containing amino acids. Each subunit includes a heme domain and a molybdopterin-binding domain, catalyzing the oxidation of sulfite to sulfate. The enzyme itself is a single polypeptide chain with a molecular mass of approximately 53.9 kDa, as seen in recombinant forms produced in E. coli. The SUOX gene provides instructions for making this enzyme, and mutations in this gene can lead to isolated sulfite oxidase deficiency (ISOD), a severe disorder causing brain damage and often fatal in early life due to the accumulation of toxic sulfite compounds in the nervous system.