These findings indicate widespread neuropathology and focused axonal neurodegeneration in alpha-galactosidase A-deficient mouse brain in association with disruption of the autophagy–lysosome pathway and may offer the basis for future mechanistic assessment of the contribution of the autophagy–lysosome pathway to neuropathologic phenotype of AFD [96]. The gene discussed is GLA; the disease is Nager acrofacial dysostosis.