Figure 1.
CRL3 ubiquitin ligase complex and its role in animal models of 16p11.2 deletion or duplication. (A) A typical CRL3 ubiquitin ligase complex comprises three subunits, including Cul3, Rbx1, and BTB proteins. Cul3 serves as the core component of the complex, which interacts with Rbx1 and E2 at the C-terminus to facilitate the transfer of ubiquitin from E2 to the substrate. At the N-terminus, Cul3 interacts with various BTB proteins, which recognizes and recruits different substrates for ubiquitination and degradation. This complex plays a crucial role in several diseases, such as cancers, neurodevelopmental disorders, and cardiovascular diseases. (B) Deletion or duplication of the 16p11.2 CNV leads to a decrease or increase in KCTD13 levels, respectively. The KCTD13-Cul3-RhoA pathway regulates RhoA ubiquitination and degradation.
