In addition, recent reports, including ours, suggest that mutations at other sites than p.Met41 can cause VEXAS syndrome, highlighting the existence of disease mechanisms distinct from the canonical loss of cytoplasmic UBA1 activity, but molecular details have remained elusive (Faurel et al, 2023; Poulter et al, 2021; Sakuma et al, 2023; Stiburkova et al, 2023). The gene discussed is UBA1; the disease is VEXAS syndrome.