Prohibitin 1 is essential to preserve mitochondria and myelin integrity in Schwann cells

Gustavo Della-Flora Nunes, Emma R. Wilson, Leandro N. Marziali, Edward Hurley, Nicholas Silvestri, Bin He, Bert W. O’Malley, Bogdan Beirowski, Yannick Poitelon, Lawrence Wrabetz and M. Laura Feltri ()
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Gustavo Della-Flora Nunes: University at Buffalo
Emma R. Wilson: University at Buffalo
Leandro N. Marziali: University at Buffalo
Edward Hurley: University at Buffalo
Nicholas Silvestri: University at Buffalo
Bin He: Houston Methodist Hospital
Bert W. O’Malley: Baylor College of Medicine
Bogdan Beirowski: University at Buffalo
Yannick Poitelon: Albany Medical College, Dept of Neuroscience and Experimental Therapeutics
Lawrence Wrabetz: University at Buffalo
M. Laura Feltri: University at Buffalo

Nature Communications, 2021, vol. 12, issue 1, 1-16

Abstract: Abstract In peripheral nerves, Schwann cells form myelin and provide trophic support to axons. We previously showed that the mitochondrial protein prohibitin 2 can localize to the axon-Schwann-cell interface and is required for developmental myelination. Whether the homologous protein prohibitin 1 has a similar role, and whether prohibitins also play important roles in Schwann cell mitochondria is unknown. Here, we show that deletion of prohibitin 1 in Schwann cells minimally perturbs development, but later triggers a severe demyelinating peripheral neuropathy. Moreover, mitochondria are heavily affected by ablation of prohibitin 1 and demyelination occurs preferentially in cells with apparent mitochondrial loss. Furthermore, in response to mitochondrial damage, Schwann cells trigger the integrated stress response, but, contrary to what was previously suggested, this response is not detrimental in this context. These results identify a role for prohibitin 1 in myelin integrity and advance our understanding about the Schwann cell response to mitochondrial damage.

Date: 2021
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DOI: 10.1038/s41467-021-23552-8

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