Sumoylation tunes actin dynamics in dendritic spines by unlocking the autoinhibitory interaction between the BAR and GAP domains of OPHN1
Alessia Seccia, Marta Busnelli, Cristina Guglielmetti, Marco Erreni, Marie Pronot, Fabrizia Claudia Guarnieri, Ninoska Polona Diestra, Marta Prieto, Chiara Scacchi, Gwenola Poupon, Silvia Bassani, Davide Pozzi, Matteo Fossati, Maria Passafaro, Federico Forneris, Stèphane Martin, Michela Matteoli, Alessandra Folci · FENS Forum 2024
Ophn1 gene encodes Oligophrenin-1 (OPHN1), a Rho-GAP protein highly expressed in neurons. In humans, all Ophn1 mutations cause the loss of function of OPHN1 leading to syndromic intellectual disability (ID). In neurons, OPHN1 regulates dendritic spine density and architecture, actin dynamics and AMPA receptor (AMPAR) trafficking. Interestingly, we identified for the first time OPHN1 as a novel target of sumoylation. Sumoylation is a post-translational modification essential to the modulation of several neuronal functions, including neurotransmitter release and synaptic plasticity. Altered sumo
ePoster · Neuroscience