OPA1 and Leber hereditary optic neuropathy: In this regard, previous studies on animal models, have shown that OPA1 gene variants causing loss of GCs, was accompanied by activation of astrocytes.52,53 Also in LHON patients, the mitochondrial complex I dysfunction in the retina has been shown to trigger an innate immune and inflammatory response that in addition to the loss of retinal GC, results in astrocyte activation.54