Abstract :
[en] Cerebral cortex development involves the sequential progression of biological steps driven by molecular pathways whose tight regulation often relies on ubiquitination. Ubiquitination is a post-translational modification involved in all aspects of cellular homeostasis through the attachment of a ubiquitin moiety on proteins. Over the past years, an increasing amount of research has highlighted the crucial role played by ubiquitin ligases in every step of cortical development and whose impairment often leads to various neurodevelopmental disorders. In this review, we focus on the key contributions of E3 ubiquitin ligases for the progression of the different steps of corticogenesis, as well as the pathological consequences of their mutations, often resulting in malformations of cortical development. Finally, we discuss some promising targeted treatment strategies for these diseases based on recent advances in the field.
Scopus citations®
without self-citations
0