MSF01 Frataxin monoclonal antibody


$295.00 (100 g)

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This product can also be ordered as part of a sample pack.



Brief Description & Uses

100 g of monoclonal antibody against frataxin, suitable for use in Western Blotting and Immunocytochemistry. Isolated mitochondria are available from several species for use as control samples. To see the complete list of control samples available from MitoSciences, click here.

Frataxin is a 17 kDa nuclear-encoded mitochondrial protein. In humans the gene is localized on chromosome 9 and is highly conserved during evolution. The gene is expressed in every cell, although in varying levels in different tissues and during development.

The specific function of frataxin is still unknown, but it has been shown to play a role in iron metabolism. Studies have demonstrated that the deletion of the frataxin gene in yeast results in iron accumulation in mitochondria and loss of respiration. Recombinant human frataxin has been shown to bind iron in vitro, and increased mitochondrial iron levels have been observed in patients with Friedreich's Ataxia (FRDA).

FRDA is an inherited recessive neurodegenerative disorder caused by decreased expression levels of frataxin. The most common mutation causing FRDA is the expansion of the GAA triplet repeat. Disease-associate repeats have 100 to 1700 triplets within the first intron of both copies of the gene.


Specifications

Product: Frataxin monoclonal antibody
Catalog #: MSF01
Lot #:
Clone ID: 18A5DB1
Host Species and Isotype: Mouse IgG1, κ
Immunogen: Human frataxin (full length mature form, residues 56-210)
Concentration: 1 mg/ml in HEPES-Buffered Saline (HBS) with 0.02% azide as a preservative.
Purity: Near homogeneity as judged by SDS-PAGE. The antibodies were produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Suggested Working Concentration: 3-5 g/ml for Western blotting (a chemiluminescent detection system is highly recommended); 0.5 g/ml for immunocytochemistry
Applications: Western blotting, immunocytochemistry
Antigen Species Cross-Reactivity: human
Storage: 4C
Country of Origin: USA



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Images

Downloadable Information
MSF01 Specifications Western Blotting Protocol Immunocytochemistry Protocol


Additional Information
Product Images
Product References
Protein References


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Secondary Antibodies
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