Frataxin

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Frataxin is a small protein, localized to the mitochondrion. The function of frataxin is not entirely clear, but it seems to be involved in assemby of iron-sulfur clusters.

Deficiency of frataxin is the cause of Friedrich's ataxia, a hereditary trinucleotide repeat disorder.

The three-dimensional structure of frataxin has been determined by X-ray crystallography and is available at the Protein Data Bank (entry 1EKG).

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