frataxin, ELISA Kit

Mouse Frataxin; mitochondrial (FXN) ELISA kit; CyaY; FA; FARR; FRDA; MGC57199; X25; ; frataxin

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of FXN elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9318033 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the frataxin (FXN) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing FXN. The ELISA analytical biochemical technique of the MBS9318033 kit is based on FXN antibody-FXN antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect FXN antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, FXN. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

22,924 Da

frataxin

frataxin, mitochondrial; Friedreich ataxia

Frataxin, mitochondrial

Frda; Fxn  [Similar Products]

FRDA_MOUSE

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; EC 1.16.3.1; Mitochondrial

Cellular Component: mitochondrion; cell; cytoplasm; cytosol

Molecular Function: ferroxidase activity; 2 iron, 2 sulfur cluster binding; enzyme binding; ferric iron binding; metal ion binding; ferrous iron binding; iron-sulfur cluster binding; oxidoreductase activity

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

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