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

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 axon-Schwann-cell interface is required for developmental myelination. Whether homologous 1 has a similar role, whether prohibitins also play important roles in cell mitochondria unknown. Here, we show deletion of minimally perturbs development, but later triggers severe demyelinating neuropathy. Moreover, are heavily affected by ablation demyelination occurs preferentially with apparent loss. Furthermore, response damage, trigger integrated stress response, but, contrary what was suggested, this not detrimental context. These results identify role integrity advance our understanding about damage.