亚硫酸盐氧化酶抗体Sulfite oxidase

订购亚硫酸盐氧化酶抗体Sulfite oxidase及了解更多信息可以客服人员。


亚硫酸盐氧化酶抗体Sulfite oxidase主要参数>>>

中文名称：  亚硫酸盐氧化酶抗体
英文名称：  Sulfite oxidase
抗体别名：  SO; EC 1.8.3.1; mitochondrial; Sulfite oxidase; Sulfite oxidase mitochondrial; Sulfite oxidase, mitochondrial precursor; Suox; SUOX_HUMAN.
抗体来源：  Rabbit
克隆类型：  Polyclonal
交叉反应：  Human，Mouse，Rat，Dog，Horse，
产品应用：  WB=1:100-500ELISA=1:500-1000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500
抗体浓度：  1mg/1ml
抗体性状：  Lyophilized or Liquid
免 疫 原：   KLH conjugated synthetic peptide derived from human Sulfite oxidase
分 子 量：   52kDa
抗体亚型：  IgG
纯化方法：  affinity purified by Protein A
细胞定位：  细胞浆细胞膜线粒体
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  
保存方法：
0.01M TBS(pH7.4) with 1% BSA， 0.03% Proclin300 and 50% Glycerol.
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.

Gene ID:
6821

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.{bios_back}{anti_back}{tongyogn_back}
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