The use of a mutant Nr2f1 mouse model to explore this hypothesis provided concordant observations, confirming that the loss of RGCs and axonal misguidance in early development leads to ONH and the development of optic atrophy, resulting in impaired visual acuity, and with the severity of the structural defects depending on the residual amount of wild-type protein. Here, NR2F1 is linked to hereditary optic atrophy.