Given mitochondrial dysfunction and preferential loss of RGCs observed in FD patients and mouse models [6,7,8,9,10], in conjunction with energetic deficits revealed in the analysis of FD patients’ stool and serum metabolite profiles [20,21], we sought to characterize temporal changes in the retinal metabolome as a function of the progressive neurodegeneration observed in the Pax6-Cre; Elp1LoxP/LoxP FD mouse model. This evidence concerns the gene PAX6 and Fabry disease.