Polyclonal Antibody to Frataxin (FXN) | MyBioSource MBS2027065 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Polyclonal Antibody to Frataxin (FXN)

catalog :

MBS2027065

quantity :

0.01 mg

price :

110 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

mouse

application :

western blot, immunohistochemistry, immunocytochemistry, immunoprecipitation

more info or order :

MyBioSource product webpage

image

image 1 :

Western Blot: Sample: Recombinant Frataxin, Mouse.

image 2 :

DAB staining on fromalin fixed paraffin- embedded kidney tissue)

product information

catalog number :

MBS2027065

products type :

Antibody

products full name :

Polyclonal Antibody to Frataxin (FXN)

products short name :

[Frataxin]

other names :

[frataxin, mitochondrial; Frataxin, mitochondrial; frataxin, mitochondrial; frataxin]

products gene name :

[FXN]

other gene names :

[Fxn; Fxn; FA; X25; FARR; Frda; Frda; Fxn]

clonality :

polyclonal

host :

rabbit

purity :

Antigen-specific affinity chromatography followed by Protein A affinity chromatography

form :

Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3, 50% glycerol.

concentration :

1 mg/ml

storage stability :

Storage: Avoid repeated freeze/thaw cycles. Store at 4ºC for frequent use. Aliquot and store at -20ºC for 24 months. Stability Test: 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.

tested application :

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC), Immunoprecipitation (IP)

app notes :

Western blotting: 0.5-2ug/mL;. Immunohistochemistry: 5-20ug/mL;. Immunocytochemistry: 5-20ug/mL;. Optimal working dilutions must be determined by end user.

image1 heading :

Western Blot (WB)

image2 heading :

Immunohistochemistry (IHC)

other info1 :

Organism Species: Mus musculus (Mouse). Source: Polyclonal antibody preparation. Traits: Liquid. Immunogen: Recombinant FXN (Leu41~Thr207) expressed in E.coli

other info2 :

Conjugated Antibody: The APC conjugated antibody version of this item is also available as catalog #MBS2066129

ncbi gi num :

6679863

ncbi acc num :

NP_032070.1

ncbi gb acc num :

NM_008044.2

uniprot acc num :

O35943

uniprot summary :

FXN: 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. Defects in FXN are the cause of Friedreich ataxia (FRDA). FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. Belongs to the frataxin family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: EC 1.16.3.1; Mitochondrial; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 19 19 B. Cellular Component: cytoplasm; cytosol; mitochondrion. Molecular Function: 2 iron, 2 sulfur cluster binding; enzyme binding; ferric iron binding; ferrous iron binding; ferroxidase activity; iron-sulfur cluster binding; metal ion binding; oxidoreductase activity. Biological Process: adult walking behavior; aerobic respiration; cellular iron ion homeostasis; embryonic development ending in birth or egg hatching; heme biosynthetic process; ion transport; iron incorporation into metallo-sulfur cluster; iron ion homeostasis; iron-sulfur cluster assembly; mitochondrion organization; negative regulation of apoptosis; negative regulation of multicellular organism growth; negative regulation of organ growth; oxidative phosphorylation; positive regulation of axon extension; positive regulation of catalytic activity; positive regulation of cell growth; positive regulation of cell proliferation; positive regulation of lyase activity; proprioception; protein autoprocessing; response to iron ion; transport

size1 :

0.01 mg

price1 :

110 USD

size2 :

0.02 mg

price2 :

130

size3 :

0.05 mg

price3 :

185

size4 :

0.1 mg

price4 :

230

size5 :

0.2 mg

price5 :

345

size6 :

1 mg

price6 :

825

more info or order :

MyBioSource product webpage