Rabbit anti-Human FXN Polyclonal Antibody | anti-FXN antibody

FXN, CT (Frataxin, Mitochondrial, Friedreich Ataxia Protein, Frataxin Intermediate Form, Frataxin(56-210), Frataxin(81-210), FRDA, X25) (PE)

Cat. Num. AAA6476895

• Gene Names: FXN; FA; X25; CyaY; FARR; FRDA
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: FXN; Polyclonal Antibody; CT (Frataxin; Mitochondrial; Friedreich Ataxia Protein; Frataxin Intermediate Form; Frataxin(56-210); Frataxin(81-210); FRDA; X25) (PE); anti-FXN antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human FXN.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-FXN antibody: Immunohistochemistry (IHC), FLISA, Western Blot (WB)
• Application Notes: IHC: 1:10-1:50; FLISA: 1:1,000; WB: 1:100-1:500; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the C-terminal region of human FXN (KLH).
• Conjugate: PE
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of anti-FXN Antibody (C-term) in 293 cell line lysates (35ug/lane). FXN (arrow) was detected using the purified Pab.)

Western Blot (WB)

(Western blot analysis of FXN (arrow) using rabbit polyclonal FXN Antibody (C-term) MBS627773. 293 cell lysates (2ug/lane) either nontransfected (Lane 1) or transiently transfected with the FXN gene (Lane 2) (Origene Technologies).)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human lung carcinoma tissue reacted with FXN antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining.)

Product Categories/Family for anti-FXN antibody

Antibodies; Mitochondrial Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 31077081
• NCBI GeneID: 2395
• NCBI Accession #: NP_000135
• NCBI GenBank Nucleotide #: NM_000144.4
• UniProt Accession #: Q16595; O15545; O95656; Q15294; Q5VZ01; A8MXJ6; C9JJ89
• Molecular Weight: 21,416 Da
• NCBI Official Full Name: frataxin, mitochondrial isoform 1 preproprotein
• NCBI Official Synonym Full Names: frataxin
• NCBI Official Symbol: FXN
• NCBI Official Synonym Symbols: FA; X25; CyaY; FARR; FRDA
• NCBI Protein Information: frataxin, mitochondrial
• UniProt Protein Name: Frataxin, mitochondrial
• UniProt Gene Name: FXN
• UniProt Synonym Gene Names: FRDA; X25; Fxn; i-FXN
• UniProt Entry Name: FRDA_HUMAN

NCBI Description

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]

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: Motility/polarity/chemotaxis; Mitochondrial; EC 1.16.3.1

Chromosomal Location of Human Ortholog: 9q21.11

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function: 2 iron, 2 sulfur cluster binding; ferroxidase activity; 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 metalloenzyme activity; positive regulation of transferase activity; proprioception; 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; negative regulation of apoptosis; positive regulation of oxidoreductase activity; oxidative phosphorylation; heme biosynthetic process

Disease: Friedreich Ataxia 1