产品详情
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.
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