FIGURE 2.

Innate immune response cells and molecules in the pathogenesis of ADPKD. In this scheme, we describe the major innate immune cells in the pathogenesis of ADPKD, including macrophages (referred to M2), NK, NKT and γδ T cells, and the major molecules that participate in this process, including DAMPs, glycolipids, and cytokines. We indicate the main receptors on immune cells, such as MHC I/II on macrophages, TCRs and NKRs on NK, NKT and γδ T cells. Pkd1 deficient renal cells release Mcp-1 and Cxcl16, which recruit macrophages to the kidney, other cytokines also attract NK, NKT and γδ T cells to infiltrate to kidney as well. Activated macrophages release TNF-α and other cytokines that stimulate renal cell proliferation and induce stress response, resulting in the accumulation of DAMPs in fluid or intestinal or induction of MICA/B on surface of renal cells. Upon recognition of DAMPs and glycolipids released by damaged or dying cells, or MICA/B presented on the surface of cystic cells, activated macrophages, NK, NKT and γδ T cells produce cytokines, such as TNF-α, IFN-ɣ, and TGF-β etc, to further promote cystic renal cell proliferation or renal fibrosis in ADPKD.