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Data sheet of Mouse Monoclonal antibody to human Frataxin
| Brand | ProteoGenix |
| Product type | Primary antibodies |
| Reactivity | Human |
| Clonality | Monoclonal |
| Host species | Mouse |
| Applications | E, WB, IC |
More info about Mouse Monoclonal antibody to human Frataxin
| Brand: | ProteoGenix |
| Proteogenix reference: | PTGX-FRA-1G2 |
| Product type: | Primary antibodies |
| Clonality: | Monoclonal |
| Product name: | Mouse Monoclonal antibody to human Frataxin |
| Host species: | Mouse |
| Target species: | Human |
| Reactivity: | Human |
| Applications: | E, WB, IC |
| Size: | 100µl |
| Clone name: | 2FRAâ€1G2 |
| Uniprot: | #N/A |
| Format: | Crude Ascites fluid |
| Immunogen: | TrpE-Frataxin full length |
| Specificity: | Human Frataxin |
| Dilution: | 1/5000 for western blot 1/100-1/1000 for immunofluorescence |
| Alternative names: | CyaY antibody, d-FXN antibody, FA antibody, FARR antibody, Frataxin mature form antibody, Frataxin antibody, FRDA antibody, FRDA_HUMAN antibody, Friedreich ataxia protein antibody, Fxn antibody, i-FXN antibody, m56-FXN antibody, m78-FXN antibody, m81-FXN antibody, MGC57199 antibody, X25 antibody |
| Isotype: | IgG1, k |
| Form: | liquid/frozen |
| Spec:conjugate/tag/label: | Unconjugated |
| Research area: | Cancer, Cell Type or Organelle Marker, Epigenetics & Nuclear Signalling, Metabolism, Neurobiology |
| Background information: | Mouse monoclonal anti-frataxin, clone 2FRA-1G2 recognize human Frataxin. Frataxin is a monomeric mitochondrial protein that in humans is encoded by the FXN gene. The specific function of Frataxin is still unknown but it seems to be involved in assembly of iron-sulfur clusters. Many researchers have demonstrated that the deletion of the Frataxin gene in yeast results in iron accumulation in mitochondria and loss of respiration. Frataxin mRNA is mainly expressed in tissues with a huge metabolic rate. ). Mouse and yeast Frataxin homologues have a potential N-terminal mitochondrial targeting sequence, and human Frataxin has been discovered to co-localise with a mitochondrial protein. Besides, disruption of the yeast gen has been shown to result in mitochondrial dysfunction. Reduced expression of frataxin is the cause of Friedreich's ataxia (FRDA), a lethal neurodegenerative disease caused by a mutation in the nuclear genome. |
| Publications: | 1. Campuzano & al Science 271 : 1423-1427 2. Bidichandani & al Am J Jum Genet 62 : 111-121 3. Campuzano & al Hum Mol Genet 6 : 1771-1780 4. Babcock & al Science 276 : 1709-1712 5. Foury & al FEBS Lett 411 : 373 –377 6. Lamarche JB Lectenberg R Handbook of cerebellar diseases, Marcel Dekker, New York p.453-458 7. Rôtig & al Nat Genet 17 : 215-217 8. Kournikova & al Hum Mol Genet 7 : 1845-1849 |
| Storage: | Store at -20°C. Aliquot to avoid repeated freezing and thawing. |
| Delivery lead time in business days in europe: | 2-3 |
| Delivery conditions: | Dry Ice |
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