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货号： bs-10426R-AF488 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-10426R-AF488
- 英文名称
- Anti-Sulfite oxidase/AF488
- 中文名称
- AF488标记的亚硫酸盐氧化酶抗体
- 别 名
- SO; EC 1.8.3.1; mitochondrial; Sulfite oxidase; Sulfite oxidase mitochondrial; Sulfite oxidase, mitochondrial precursor; Suox; SUOX_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 神经生物学 信号转导 转录调节因子 新陈代谢 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 52kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Sulfite oxidase
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. [provided by RefSeq, Jul 2008]
Subunit:
Homodimer.
Subcellular Location:
Mitochondrion intermembrane space.
DISEASE:
Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD) [MIM:272300]; also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.
Similarity:
Contains 1 cytochrome b5 heme-binding domain.
Database links:Entrez Gene: 6821 Human
Entrez Gene: 211389 Mouse
Omim: 606887 Human
SwissProt: P51687 Human
SwissProt: Q8R086 Mouse
Unigene: 558403 Human
Unigene: 23352 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.