Phagocytic cell death leads to enhanced release of pro-inflammatory S100A12 in familial Mediterranean fever.

ABSTRACT:

Background

Familial Mediterranean fever (FMF) is a prototypical autoinflammatory syndrome associated with phagocytic cell activation. Pyrin mutations are the genetic basis of this disease, and its expression has been shown in monocytes, granulocytes, dendritic cells, and synovial fibroblasts. Pyrin functions as a cytosolic pattern recognition receptor and forms a distinct pyrin inflammasome. The phagocyte-specific protein S100A12 is predominantly expressed in granulocytes and belongs to the group of damage associated molecular patterns (DAMP). S100A12 can be detected at massively elevated levels in the serum of FMF patients, even in clinically inactive disease. Whether this is crucial for FMF pathogenesis is as yet unknown, and we therefore investigated the mechanisms of S100A12 re

SUBMITTER: Varga G 

PROVIDER: S-EPMC10716088 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature