,30,31 Pathogenic variants in AP genes are rarely detected in patients with monoclonal gammopathy–associated C3G21,64; rather, monoclonal Ig (MIg) may lead to overactivation of the AP by directly activating C3 convertase or by acting as autoantibodies against other regulatory complement proteins.21 Here, DHCR7-DT is linked to monoclonal gammopathy.