BD-PM3073-FH(7F1)Mouse Monoclonal Antibody-现货抗体产品库武汉枢密脑科学技术有限公司

FH(7F1)Mouse Monoclonal Antibody

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产品基本信息

产品货号

BD-PM3073

产品名称

FH(7F1)Mouse Monoclonal Antibody

别名

Fumarate hydratase, mitochondrial (Fumarase) (EC 4.2.1.2)

类别

常规抗体

基因名称

蛋白名称

Fumarate hydratase, mitochondrial

推荐应用

反应种属

Human,Mouse,Rat

存储缓冲液

PBS, pH 7.4, containing 0.5%BSA, 0.02% New type preservative N as Preservative and 50% Glycerol.

Human Gene ID

2271

Human Gene Link

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2271

Human Swissprot No.

P07954

Human Swissprot Link

http://www.uniprot.org/uniprotkb/P07954/entry

Mouse Gene ID

14194

Mouse Gene Link

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=14194

Mouse Swissprot No.

P97807

Mouse Swissprot Link

http://www.uniprot.org/uniprot/P97807

Rat Swissprot No.

P14408

Rat Swissprot Link

http://www.uniprot.org/uniprot/P14408

免疫原

Synthetic Peptide of FH

特异性

The antibody detects endogenous FH proteins.

稀释度

WB 1:3000 IF 1:200 IHC 1:50-300

预测分子量

50kD

运输及保存条件

-20°C/1 year

宿主

Monoclonal, Mouse

背景介绍

The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008],

组织表达

Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.

细胞定位

[Isoform Mitochondrial]: Mitochondrion .; [Isoform Cytoplasmic]: Cytoplasm, cytosol . Nucleus . Chromosome . Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs) following phosphorylation by PRKDC. .

信号通路

Citrate cycle (TCA cycle);Pathways in cancer;Renal cell carcinoma;

功能

catalytic activity:(S)-malate = fumarate + H(2)O.,disease:Defects in FH are the cause of fumarase deficiency (FD) [MIM:606812]; also known as fumaricaciduria. FD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.,disease:Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].,disease:Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).,function:Also acts as a tumor suppressor.,miscellaneous:There are 2 substrate binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors.,pathway:Carbohydrate metabolism; tricarboxylic acid cycle.,PTM:Isoform Cytoplasmic is acetylated at position 2.,similarity:Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.,subunit:Homotetramer.,

期货

现货

纯化

The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.

Immunohistochemical analysis of paraffin-embedded Human-uterus tissue. 1,FH Monoclonal Antibody（7F1） was diluted at 1:200（4°C,overnight）. 2, Sodium citrate pH 6.0 was used for antibody retrieval（>98°C,20min）. 3,Secondary antibody was diluted at 1:200（room tempeRature, 30min）. Negative control was used by secondary antibody only.

Immunohistochemical analysis of paraffin-embedded Rat-heart tissue. 1,FH Monoclonal Antibody（7F1） was diluted at 1:200（4°C,overnight）. 2, Sodium citrate pH 6.0 was used for antibody retrieval（>98°C,20min）. 3,Secondary antibody was diluted at 1:200（room tempeRature, 30min）. Negative control was used by secondary antibody only.

Immunohistochemical analysis of paraffin-embedded Mouse-heart tissue. 1,FH Monoclonal Antibody（7F1） was diluted at 1:200（4°C,overnight）. 2, Sodium citrate pH 6.0 was used for antibody retrieval（>98°C,20min）. 3,Secondary antibody was diluted at 1:200（room tempeRature, 30min）. Negative control was used by secondary antibody only.

Immunofluorescence analysis of Human-liver-cancer tissue. 1,FH Monoclonal Antibody（7F1）（red） was diluted at 1:200（4°C,overnight）. 2, Cy3 labled Secondary antibody was diluted at 1:300（room temperature, 50min）.3, Picture B: DAPI（blue） 10min. Picture A:Target. Picture B: DAPI. Picture C: merge of A+B

Immunofluorescence analysis of Mouse-testis tissue. 1,FH Monoclonal Antibody（7F1）（red） was diluted at 1:200（4°C,overnight）. 2, Cy3 labled Secondary antibody was diluted at 1:300（room temperature, 50min）.3, Picture B: DAPI（blue） 10min. Picture A:Target. Picture B: DAPI. Picture C: merge of A+B

Western blot analysis of 1） 293T, 2） HepG2, 3） Hela, diluted at 1:3000.