Biochemistry of Autoinflammatory Diseases: Catalyzing Monogenic Disease.

ABSTRACT: Monogenic autoinflammatory disorders are a group of conditions defined by systemic or localized inflammation without identifiable causes, such as infection. In contrast to classical primary immunodeficiencies that manifest with impaired immune responses, these disorders are due to defects in genes that regulate innate immunity leading to constitutive activation of pro-inflammatory signaling. Through studying patients with rare autoinflammatory conditions, novel mechanisms of inflammation have been identified that bare on our understanding not only of basic signaling in inflammatory cells, but also of the pathogenesis of more common inflammatory diseases and have guided treatment modalities. Autoinflammation has further been implicated as an important component of cardiovascular, neurodegen

SUBMITTER: Beck DB 

PROVIDER: S-EPMC6365650 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature