Cofilin dysregulation alters actin turnover in frataxin-deficient neurons

Diana C. Muñoz-Lasso, Belén Mollá, Pablo Calap-Quintana, José Luis García-Giménez, Federico V. Pallardo, Francesc Palau & Pilar Gonzalez-Cabo; Sci Rep 10, 5207 (2020). https://doi.org/10.1038/s41598-020-62050-7.

In this work, we demonstrate the dysregulation of the actin cytoskeleton in frataxin-deficient neurites of DRG neurons from the YG8R mice as a result of hyperactivation of cofilin-1 and the complex ARP2/3, which could affect the dynamics of growth cones and neurite growth. Recent work has determined that cofilin hyperactivation is age-dependent57, so it would be interesting to analyze these results at the embryonic stage. Assessing if there is a dysregulation of cofilin that could contribute to a failure in the neurite growth of embryonic neurons would help to understand the hypoplastic phenomena previously described in FRDA patients1. As a whole, our results show for the first time an imbalance in the activity of cofilin that could explain, at least partially, the neuropathy of FRDA. Future research will determine if cofilin is a potential molecular target for a therapeutic approach in FRDA.

Cofilin dysregulation alters actin turnover in frataxin-deficient neurons