Receptor-interacting serine/threonine-protein kinase 1 (RIPK1) is a key mediator of cell
death and inflammation. The unique hydrophobic pocket in the allosteric regulatory domain …

Molecular, cellular, and clinical studies of human inborn errors of immunity have
revolutionized our understanding of their pathogenesis, considerably broadened their …

Background Adult-onset inflammatory syndromes often manifest with overlapping clinical
features. Variants in ubiquitin-related genes, previously implicated in autoinflammatory …

Ubiquitylation is an essential post-translational modification that regulates intracellular
signalling networks by triggering proteasomal substrate degradation, changing the activity of …

Fifty years have elapsed since the term common variable immunodeficiency (CVID) was
introduced to accommodate the many and varied antibody deficiencies being identified in …

Objective Somatic mutations in UBA1 have recently been causally linked to a severe adult‐
onset inflammatory condition referred to as VEXAS (vacuoles, E1 enzyme, X‐linked …

Tumor necrosis factor-alpha (TNF-α) is a pleiotropic immune cytokine that belongs to the
TNF superfamily of receptor ligands. The cytokine exists as either a transmembrane or a …

Immune cell activation is a stringently regulated process, as exaggerated innate and
adaptive immune responses can lead to autoinflammatory and autoimmune diseases …

Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphohistiocytosis
(HLH), is a cytokine storm syndrome associated with multi-organ system dysfunction and …

Vasculitis is an inflammatory disorder of the blood vessels that causes damage to a wide
variety of organs through tissue ischemia. Vasculitis is classified according to the size (large …