Mouse anti-Human, Mouse Frataxin Monoclonal Antibody | anti-FXN antibody

Frataxin (FXN)

Cat. Num. AAA602148

• Gene Names: FXN; FA; X25; CyaY; FARR; FRDA; MGC57199; FXN
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot, Immunofluorescence
• Purity: Ascites; Ascites
• Synonyms: Frataxin; Monoclonal Antibody; Frataxin (FXN); Anti -Frataxin (FXN); anti-FXN antibody

• Host: Mouse
• Reactivity: Human, Mouse
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: 2Q54
• Specificity: Recognizes only isoforms of human Frataxin containing exon 4. On Western Blots of normal human muscle, heart, cerebellum, and spinal cord extracts, antibody recognizes a band migrating at ~18kD corresponding to processed human frataxin (Campuzano, 1997). Slight cross reactivity with myosin may be observed by Western Blot. Immunofluorescent labeling of HeLa cells with F6100 demonstrates that frataxin is predominantly localized in the mitochondria (Campuzano, 1997). Species Crossreactivity: mouse.
• Purity/Purification: Ascites; Ascites
• Form/Format: Supplied as a liquid in PBS, 40% glycerol. No preservatives added.

• Applicable Applications for anti-FXN antibody: ELISA (EL/EIA), Western Blot (WB), Immunofluorescence (IF)
• Application Notes: Suitable for use in Western Blot, Immunofluorescence and ELISA.; Dilution: Western Blot (Natural and recombinant protein): 1:2500; Immunofluorescence: 1:50-1:500 Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody.
• Immunogen: TrpE-Frataxin full length. Epitope: Maps to the first part of exon 4 of frataxin.
• Preparation and Storage: May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Testing Data

(PC12 lysate was resolved by electrophoresis, transferred to PVDF membrane and probed with Frataxin (1:1000 dilution of this lot). Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and a chemiluminescence detection system. Arrow indicates protein Frataxin (~18kD))

Related Product Information for anti-FXN antibody

Frataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle. Frataxin is expressed as a 30kD precursor that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase to yield the mature protein. The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20kD (aa 42-210). Further cleavage of the N-terminus of this 20kD intermediate by MPP results in the mature 18kD frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich's ataxia, an autosomal recessive, progressive degenerative disease. In the majority of cases of Friedreich's ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription. This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress.

Product Categories/Family for anti-FXN antibody

Antibodies; Abs to Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 4100063
• NCBI GeneID: 2395
• UniProt Accession #: O96034; Q16595
• Molecular Weight: 7,201 Da
• NCBI Official Full Name: frataxin
• NCBI Official Synonym Full Names: frataxin
• NCBI Official Symbol: FXN
• NCBI Official Synonym Symbols: FA; X25; CyaY; FARR; FRDA; MGC57199; FXN
• NCBI Protein Information: frataxin, mitochondrial; OTTHUMP00000215688; friedreich ataxia protein
• UniProt Protein Name: Frataxin
• Protein Family: Frataxin
• UniProt Gene Name: x25
• UniProt Entry Name: O96034_HUMAN

NCBI Description

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]

Uniprot Description

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: Mitochondrial; Motility/polarity/chemotaxis; EC 1.16.3.1

Chromosomal Location of Human Ortholog: 9q21.11

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function: ferroxidase activity; 2 iron, 2 sulfur cluster binding; protein binding; ferric iron binding; ferrous iron binding; iron-sulfur cluster binding

Biological Process: mitochondrion organization and biogenesis; negative regulation of multicellular organism growth; cellular iron ion homeostasis; positive regulation of transferase activity; proprioception; positive regulation of metalloenzyme activity; negative regulation of organ growth; positive regulation of cell growth; adult walking behavior; protein autoprocessing; embryonic development ending in birth or egg hatching; iron incorporation into metallo-sulfur cluster; positive regulation of lyase activity; positive regulation of cell proliferation; aerobic respiration; ion transport; response to iron ion; positive regulation of oxidoreductase activity; oxidative phosphorylation; negative regulation of apoptosis; heme biosynthetic process

Disease: Friedreich Ataxia 1