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Western blot data are representative of 2 independent experiments

Peer review Peer review information Nature Chemistry thanks Chad Elmore, Da-Gang Yu and the other, anonymous, reviewer(s) for their contribution to the peer review of this work

The authors also would like to thank the Imp@ct core facility (SFR Bonamy, Nantes Universit, CNRS, Inserm, Nantes, France), member of the Scientific Interest Group Biogenouest, for technical support

A nuclear defect in the 4p16 region predisposes to multiple mitochondrial DNA deletions in families with Wolfram syndrome

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