Anti-Fumarate hydratase抗体Defects in FH are the cause of fumarase deficiency （FHD）also known as fumaricaciduria.

产品编号 yb-3938R
英文名称 Anti-Fumarate hydratase抗体
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.
 Anti-Fumarate hydratase抗体
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 IHC-P=1:100-500 IHC-F=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 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 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.
 
PubMed PubMed
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.