$250.00

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Brief Description & Uses

100 µg of monoclonal antibody against frataxin, suitable for use in Western Blotting and Immunocytochemistry. Human heart mitochondria are also included as a Western Blotting control.

Frataxin is a mitochondrial protein that is approximately 17 kDa in size in its monomeric form. The gene encoding frataxin in humans lies on chromosome 9. The codon GAA is repeated 100-1700 times in both copies of this gene in the majority of patients having Friedreich's Ataxia (FRDA). FRDA is an autosomal recessive disorder causing neurodegeneration and cardiomyopathy. FRDA patients show low expression of frataxin.

Frataxin has been implicated in mitochondrial iron homeostasis although the mechanism by which this protein is involved in iron metabolism is still not clear. Deletion of the frataxin gene in yeast results in iron accumulation in the mitochondria and loss of respiration. Increased mitochondrial iron levels have also been observed in FRDA patients. Recombinant human frataxin, both in its monomeric form and in a polymeric form, has been shown to bind iron in vitro.

Product:

Mouse monoclonal antibody anti-Frataxin

Catalog #:

MSM01

Lot #:

FCEBFH

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 antibody was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.

Suggested Working Concentration:

3-5 µg/ml for Western blotting (a chemiluminescent detection system is highly recommended), 0.5-5 µg/ml for Immunocytochemistry

Applications:

Western blotting and Immunocytochemistry

Antigen Species Cross-Reactivity:

human

Storage:

4°C

Country of Origin:

USA