ARG58327
anti-Frataxin antibody
anti-Frataxin antibody for ICC/IF,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human,Mouse,Rat
Overview
| Product Description | Rabbit Polyclonal antibody recognizes Frataxin |
|---|---|
| Tested Reactivity | Hu, Ms, Rat |
| Tested Application | ICC/IF, IHC-P, WB |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Target Name | Frataxin |
| Antigen Species | Human |
| Immunogen | Recombinant fusion protein corresponding to aa. 42-210 of Human Frataxin (NP_000135.2). |
| Conjugation | Un-conjugated |
| Alternate Names | Frataxin; Friedreich ataxia protein; d-FXN; Frataxin, mitochondrial; FRDA; i-FXN; m56-FXN; CyaY; m78-FXN; FA; Fxn; 56-210; FARR; X25; 78-210; m81-FXN; EC 1.16.3.1; 81-210 |
Application Instructions
| Application Suggestion |
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| Application Note | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||||||
| Positive Control | Raji | ||||||||
| Observed Size | 14 kDa |
Properties
| Form | Liquid |
|---|---|
| Purification | Affinity purified. |
| Buffer | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
| Preservative | 0.02% Sodium azide |
| Stabilizer | 50% Glycerol |
| Storage Instruction | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| Note | For laboratory research only, not for drug, diagnostic or other use. |
Bioinformation
| Database Links | |
|---|---|
| Gene Symbol | FXN |
| Gene Full Name | frataxin |
| Background | This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2009] |
| Function | Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1. [UniProt] |
| Cellular Localization | Cytoplasm, Mitochondrion. [UniProt] |
| Calculated MW | 23 kDa |
| PTM | Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure. [UniProt] |
