Details
| Product Name | FH |
| Chinese Name | 富马酸水合酶抗体 |
| Alias | mitochondrial; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata. |
| Research Area | Tumour Cell biology immunology transcriptional regulatory factor Mitochondrion |
| Immunogen Species | Rabbit |
| Clonality | Polyclonal |
| React Species | Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit,Sheep) |
| Applications | WB=1:500-2000 （Paraffin sections need antigen repair）
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| Theoretical molecular weight | 51kDa |
| Cellular localization | cytoplasmic Mitochondrion |
| Form | Liquid |
| Concentration | 1mg/ml |
| immunogen | KLH conjugated synthetic peptide derived from human Fumarate hydratase: 411-510/510 |
| Lsotype | IgG |
| Purification | affinity purified by Protein A |
| Buffer Solution | 1M TBS(pH7.4) with 1% BSA, 3% Proclin300 and 50% Glycerol. |
| Storage | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| Attention | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| Product Detail |
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 |
| Product Picture |
Sample:
Lane 1: Mouse Heart tissue lysates Lane 2: Mouse Cerebrum tissue lysates Lane 3: Rat Heart tissue lysates Lane 4: Rat Cerebrum tissue lysates Primary: Anti-FH (SL3938R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kDa Observed band size: 51 kDa |
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