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An integrated ADNIV disease model. (A) Histology of the CAPN5R243L uveitis model. (B) The CAPN5R243L disease allele transgene is expressed in retina photoreceptors. Because of the high calcium levels in the retina for phototransduction, CAPN5 mutations in the retina are particularly sensitive. TLR signaling and cyotokine secretion trigger retinal and iris vessel hyperpermeability and a cell-mediated autoinflammatory reaction that includes vitreous inflammation and fibrosis.
