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货号： bs-21094R-AF594 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-21094R-AF594
- 英文名称
- Anti-OPA3/AF594
- 中文名称
- AF594标记的视神经萎缩蛋白3抗体
- 别 名
- FLJ22187; FLJ25932; Iraqi Jewish optic atrophy plus (3 methylglutaconicaciduria type 3); MGA3; MGC75494; OPA 3; OPA3 protein; Optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia); Optic atrophy 3 (Iraqi Jewish optic atrophy plus); Optic atrophy 3; Optic atrophy 3 protein.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 神经生物学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Dog, Cow, Horse, Sheep,
- 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
- 20kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human OPA3
- 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:
The mouse ortholog of this protein co-purifies with the mitochondrial inner membrane. Mutations in this gene have been shown to result in 3-methylglutaconic aciduria type III and autosomal dominant optic atrophy and cataract. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
Function:
OPA3 may play some role in mitochondrial processes. Defects in OPA3 are the cause of 3-methylglutaconic aciduria type 3 (MGA3), also known as optic atrophy plus syndrome, and of optic atrophy type 3 (OPA3), also known as autosomal dominant optic atrophy and cataract (ADOAC).
Subcellular Location:
Mitochondrial.
Database links:Entrez Gene: 80207Human
Omim: 606580Human
SwissProt: Q9H6K4Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.