Lack of the Long Pentraxin PTX3 Promotes Autoimmune Lung Disease but not Glomerulonephritis in Murine Systemic Lupus Erythematosus

摘要: The long pentraxin PTX3 has multiple roles in innate immunity. For example, regulates C1q binding to pathogens and dead cells their uptake by phagocytes. It also inhibits P-selectin-mediated recruitment of leukocytes. Both these mechanisms are known be involved autoimmunity autoimmune tissue injury, e.g. systemic lupus erythematosus, but a contribution is hypothetical. To evaluate potential immunoregulatory role we crossed Ptx3-deficient mice with Fas-deficient (lpr) C57BL/6 (B6) mild lupus-like autoimmunity. was found increasingly expressed kidneys lungs B6lpr along disease progression. Lack impaired the phagocytic apoptotic T into peritoneal macrophages selectively expanded CD4/CD8 double negative while other immune cell subsets autoantibody production remained unaffected. aggravated lung disease, i.e. peribronchial perivascular CD3+ macrophage infiltrates mice. In contrast, histomorphological functional parameters nephritis unaffected Ptx3 genotype. Together, specifically suppresses that associated erythematosus. Vice versa, loss-of-function mutations gene might represent genetic risk factor for pulmonary (but not renal) manifestations or diseases.