訂購亞硫酸鹽氧化酶抗體Sulfite oxidase及了解更多信息可以客服人員。


亞硫酸鹽氧化酶抗體Sulfite oxidase主要參數>>>

中文名稱：  亞硫酸鹽氧化酶抗體
英文名稱：  Sulfite oxidase
抗體別名：  SO; EC 1.8.3.1; mitochondrial; Sulfite oxidase; Sulfite oxidase mitochondrial; Sulfite oxidase, mitochondrial precursor; Suox; SUOX_HUMAN.
抗體來源：  Rabbit
克隆類型：  Polyclonal
交叉反應：  Human，Mouse，Rat，Dog，Horse，
產品應用：  WB=1:100-500ELISA=1:500-1000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500
抗體濃度：  1mg/1ml
抗體性狀：  Lyophilized or Liquid
免 疫 原：   KLH conjugated synthetic peptide derived from human Sulfite oxidase
分 子 量：   52kDa
抗體亞型：  IgG
純化方法：  affinity purified by Protein A
細胞定位：  細胞漿細胞膜線粒體
研究領域：  腫瘤  細胞生物  神經生物學  信號轉導  轉錄調節因子  新陳代謝  線粒體  
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.
產品介紹：
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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