上海细胞库
人源细胞系| 稳转细胞系| 基因敲除株| 基因点突变细胞株| 基因过表达细胞株| 重组细胞系| 猪的细胞系| 马细胞系| 兔的细胞系| 犬的细胞系| 山羊的细胞系| 鱼的细胞系| 猴的细胞系| 仓鼠的细胞系| 狗的细胞系| 牛的细胞| 大鼠细胞系| 小鼠细胞系| 其他细胞系|
人源细胞系| 稳转细胞系| 基因敲除株| 基因点突变细胞株| 基因过表达细胞株| 重组细胞系| 猪的细胞系| 马细胞系| 兔的细胞系| 犬的细胞系| 山羊的细胞系| 鱼的细胞系| 猴的细胞系| 仓鼠的细胞系| 狗的细胞系| 牛的细胞| 大鼠细胞系| 小鼠细胞系| 其他细胞系|
| 规格 | 价格 | 库存 |
|---|---|---|
| 50ul | ￥ 1180.00 | 10 |
| 100ul | ￥ 1980.00 | 10 |
| 200ul | ￥ 2800.00 | 10 |
| 产品编号 | bsm-33167M |
| 英文名称 | Mouse?Anti-Fumarase/FH?antibody |
| 中文名称 | 富马酸水合酶单克隆抗体 |
| 别????名 | FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata.?? |
| 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 | Mouse |
| 克隆类型 | Monoclonal |
| 克?隆?号 | 4C12 |
| 交叉反应 | Human,Rat (predicted: Mouse) |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 51kDa |
| 细胞定位 | 细胞浆?线粒体 |
| 性????状 | Liquid |
| 浓????度 | 1mg/ml |
| 免?疫?原 | Recombinant human Fumarase/FH Protein? |
| 亚????型 | IgG |
| 纯化方法 | affinity purified by Protein G |
| 缓?冲?液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 | 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] Function: Also acts as a tumor suppressor. Subunit: Homotetramer. Subcellular Location: Cytoplasm, Mitochondrion Tissue Specificity: Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. DISEASE: Fumarase deficiency (FMRD): The disease is caused by mutations affecting the gene represented in this entry. A severe autosomal recessive metabolic disorder characterized by early-onset hypotonia, profound psychomotor retardation, and brain abnormalities, such as agenesis of the corpus callosum, gyral defects, and ventriculomegaly. Many patients show neonatal distress, metabolic acidosis, and/or encephalopathy. Hereditary leiomyomatosis and renal cell cancer (HLRCC): The disease is caused by mutations affecting the gene represented in this entry. A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients. Similarity: Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. SWISS: P07954 Gene ID: 2271 Database links: Entrez Gene: 2271?Human Entrez Gene: 14194?Mouse Entrez Gene: 393938?Zebrafish Omim: 136850?Human SwissProt: P07954?Human SwissProt: P97807?Mouse SwissProt: Q7SX99?Zebrafish Unigene: 592490?Human Unigene: 41502?Mouse Unigene: 29782?Rat Unigene: 104452?Zebrafish |
| 产品图片 |  Paraformaldehyde-fixed, paraffin embedded (human myocardium); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (human gastric carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (mouse heart); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (rat kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (human gastric carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.  Paraformaldehyde-fixed, paraffin embedded (human myocardium); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (bsm-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. |
