As it is the case in other animal models of MEB/WWS [41], [42], enhanced glial fibrillary acid protein (GFAP) immunoreactivity, which is reflective of astrocytic gliosis, was observed in the hippocampus and cerebral cortex of Col4a1+/Δex40 mice (Figure S2). This evidence concerns the gene COL4A1 and muscle-eye-brain disease.