Anti-FXN Rabbit pAb

Antibody type:Primary antibody

Label:Unconjugated

Modification:Unmodification

Isotype:Rabbit IgG

Host:Rabbit

Application:WB,IHC,IF

Purify method:Affinity purified

Species:Human,Mouse,Rat

Gene Name:FXN

Synonyms:FA; X25; CyaY; FARR; FRDA

Gene Synonyms:

Gene Full Name:frataxin

Gene Infomation:This nuclear gene encodes a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA from 8-33 repeats to >90 repeats results in Friedreich ataxia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2016]

Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 42-210 of human FXN (NP_000135.2).

Antigen Synonyms:Frataxin, mitochondrial

Clonality:Polyclonal antibody

Source:Human

Reaction:

Form:Liquid

Tested Applications:

• Western blot (1:100 to 1:500)
• Immunofluorescence (1:50 to 1:400)
• Immunohistochemistry (1:200 to 1:500)
• Flow cytometry analysis (1:200 to 1:500)
• Enzyme-linked Immunosorbent Assay (1:100-1:5000)

Note: Users are strongly advised to determine the optimal dilution of antibody to use for their specific applications.

Clone:

Dilution:WB1:500 -1:2000; IHC1:50 -1:200; IF1:50 -1:200(Optimal dilutions should be determined by the end user)

Mole Mass:13kDa

Location:Cytoplasm, Mitochondrion

Concentration:

Sequence Similarity:

Gene Id:

SwissProt ID:Q16595

Unigene:2395

Nucleotide Accession:

Tissue specificity:Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.

Storage:Store at -20°C. Avoid freeze / thaw cycles.

Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Background:

Molar Function:Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ 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 Fe2+ to Fe3+; 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.

Western Blot: 

Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.