András N. Spaan

Apr 23, 2024 | rupress.org | András N. Spaan

Subjects: Human disease genetics, Immunodeficiency, Innate immunity and inflammation Autoinflammation is the term used to describe disorders of hyperactivation of the immune system in the absence of autoimmunity. Around 50 monogenic inborn errors of immunity are known to underlie autoinflammatory syndromes. In an apparent paradox, autoinflammation can be associated with immunodeficiency. This paradox is illustrated by human deficiencies of the linear ubiquitin homeostasis.