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Frataxin

Molecular classification: Protein

Overview

Frataxin is a highly conserved mitochondrial protein essential for iron-sulfur cluster assembly and iron homeostasis. Deficiency leads to Friedreich's ataxia, a neurodegenerative disorder. It is primarily involved in the assembly of iron-sulfur clusters, acts as an iron chaperone, and protects against oxidative damage.

Mechanism of action

Frataxin stimulates Fe–S cluster biosynthesis by accelerating persulfide transfer. It is also involved in cellular iron homeostasis.

Biological functions

Iron-sulfur cluster assemblyIron homeostasis regulationIron chaperone activityAntioxidant activity

Disease associations

Friedreich's ataxia

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