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FARAFARA Cure FA

Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites

For iron-sulfur (Fe-S) cluster synthesis in mitochondria, the sulfur is derived from the amino acid cysteine by the cysteine desulfurase activity of Nfs1. The enzyme binds the substrate cysteine in the pyridoxal phosphate-containing site, and a persulfide is formed on the active site cysteine in a manner depending on the accessory protein Isd11.

Read More: Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites


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Jen Farmer

Jen Farmer

Executive Director

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