Reduceret eller helt manglende α- galaktosidase- aktivitet medfører akkumulation af GL- 3 i lysosomerne af mange celletyper, herunder endotel - og parenkymale celler, hvilket i sidste ende forårsager livstruende, klinisk forværring som følge af nyre -, hjerte - og cerebrovaskulære komplikationer.
Reduced or absent α - galactosidase activity results in the accumulation of GL-3 in the lysosomes of many cell types including the endothelial and parenchymal cells, ultimately leading to life-threatening clinical deteriorations as a result of renal, cardiac and cerebrovascular complications.