A4GALT and Fabry disease: Although the α-Gal A(−/0) mouse model closely resembles the underlying genetics of FD patients, alternative FD mouse models have been developed, as for example the G3Stg/GLA(−/−) mouse, which expresses human Gb3 synthase (Taguchi et al., 2013), or the NOD/SCID immune deficiency mouse that also shows tissue specific Gb3 accumulation, although without clinical manifestation (Pacienza et al., 2012).