GLA and Fabry disease: Notably, both of these subjects had very mild phenotypes, raising suspicion that the favorable skewed X-inactivation (predominantly affecting the mutant copy of GLA) observed in kidney biopsies may have also been present in other organs, especially since R112C mutation has been associated with classical Fabry disease causing ESRD [38], while case #9 in the present study had normal renal function and no history of cardiac disease or strokes.