Study finds primary cilia restrict autoinflammation by mediating PD-L1 expression

Ciliopathies are multisystem disorders characterized by the dysfunction of motile and/or non-motile cilia, which show common clinical manifestations of ciliopathies include retinal degeneration, mental retardation, renal abnormality, obesity, and skeletal dysplasia. Fibrosis of vital organs, characterized by the extensive deposition of extracellular matrix components, represents another complication frequently observed in patients and animal models of ciliopathies. However, the precise mechanism that connects ciliary defects to fibrosis remains largely elusive.

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