产品货号 :  mlR21094

英文名称 :  OPA3

中文名称 :  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.  

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 :  IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  20kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human OPA3:1-100/179

亚    型 :  IgG

纯化方法 :  affinity purified by Protein A

储 存 液 :  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

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.

PubMed :  PubMed

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.

SWISS:

Q9H6K4

Gene ID:

80207

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片