MSF01 Frataxin monoclonal antibody
$295.00 (100 µg)
Please contact us for volume discounts.
This product can also be ordered as part of a sample pack.
Brief Description & Uses
100 µg 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
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Product:
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Frataxin monoclonal antibody
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Catalog #:
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MSF01
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Lot #:
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Clone ID:
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18A5DB1
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Host Species and Isotype:
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Mouse IgG1, κ
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Immunogen:
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Human frataxin (full length mature form, residues 56-210)
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Concentration:
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1 mg/ml in HEPES-Buffered Saline (HBS) with 0.02% azide as a preservative.
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Purity:
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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.
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Suggested Working Concentration:
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3-5 µg/ml for Western blotting (a chemiluminescent detection system is highly recommended); 0.5 µg/ml for immunocytochemistry
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Applications:
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Western blotting, immunocytochemistry
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Antigen Species Cross-Reactivity:
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human
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Storage:
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4°C
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Country of Origin:
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USA
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Product References
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References for Frataxin
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