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- W4366463448 abstract "Abstract The bacterial product CNF1, through its action on the Rho GTPases, is emerging as a modulator of crucial signalling pathways involved in selected neurological diseases characterized by mitochondrial dysfunctions. Mitochondrial impairment has been hypothesized to have a key role in paramount mechanisms underlying Rett syndrome (RTT), a severe neurologic rare disorder. CNF1 has been already reported to have beneficial effects in mouse models of RTT. Using human RTT fibroblasts from four patients carrying different mutations, as a reliable disease‐in‐a‐dish model, we explored the cellular and molecular mechanisms, which can underlie the CNF1‐induced amelioration of RTT deficits. We found that CNF1 treatment modulates the Rho GTPases activity of RTT fibroblasts and induces a considerable re‐organization of the actin cytoskeleton, mainly in stress fibres. Mitochondria of RTT fibroblasts show a hyperfused morphology and CNF1 decreases the mitochondrial mass leaving substantially unaltered the mitochondrial dynamic. From a functional perspective, CNF1 induces mitochondrial membrane potential depolarization and activation of AKT in RTT fibroblasts. Given that mitochondrial quality control is altered in RTT, our results are suggestive of a reactivation of the damaged mitochondria removal via mitophagy restoration. These effects can be at the basis of the beneficial effects of CNF1 in RTT." @default.
- W4366463448 created "2023-04-22" @default.
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- W4366463448 date "2023-04-20" @default.
- W4366463448 modified "2023-10-18" @default.
- W4366463448 title "Effects of the Rho <scp>GTPase</scp>‐activating toxin <scp>CNF1</scp> on fibroblasts derived from Rett syndrome patients: A pilot study" @default.
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- W4366463448 doi "https://doi.org/10.1111/jcmm.17624" @default.
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