富马酸水合酶抗体

英文名称    Fumarate hydratase    

中文名称    富马酸水合酶抗体    

别    名    mitochondrial; FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata.    

说 明 书    0.2ml      

研究领域    肿瘤  细胞生物  免疫学  转录调节因子  线粒体      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 富马酸水合酶抗体（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    51kDa    

细胞定位    细胞浆 线粒体    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Fumarate hydratase (447-496aa)    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

富马酸水合酶抗体产品介绍    background:

Defects in FH are the cause of fumarase deficiency (FHD)also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. 

Subunit:
Homotetramer.

Subcellular Location:
Isoform Mitochondrial: Mitochondrion. Isoform Cytoplasmic: Cytoplasm.

Similarity:
Belongs to the class-II fumarase/aspartase family.

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

富马酸水合酶抗体

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