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METTL17 is an Fe-S cluster checkpoint for mitochondrial translation

Tslil Ast1, Yuzuru Itoh2, Shayan Sadre1

  • 1Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Systems Biology, Harvard Medical School, Boston, MA 02115, USA.

Molecular Cell
|January 10, 2024
PubMed
Summary

Friedreich's ataxia (FA) pathogenesis involves frataxin (FXN) depletion, impacting iron-sulfur proteins and mitochondrial translation. METTL17, a mitoribosomal factor with an iron-sulfur cluster, acts as a checkpoint for oxidative phosphorylation protein synthesis.

Keywords:
FAFe-S clusterFriedreich’s ataxiaMETTL17frataxinmitochondriamitoribosome

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