Fabry disease (FD; OMIM 301500), an LSD caused by α-galactosidase A (GLA; EC 3.2.1.22) deficiency, is characterized by the accumulation of globotriaosylceramide (Gb3) and Lyso-GB3 in the kidneys, heart, brain, and peripheral nervous system [90]. The gene discussed is GLA; the disease is Fabry disease.