We had previously crossbred asymptomatic Glatm mice15, 16 with transgenic mice expressing human Gb3 synthase (A4GALT) to generate the GlatmTg(CAG‐A4GALT) symptomatic Fabry mouse model that exhibits polyuria and renal dysfunction without any remarkable glomerular damage and provided the first clear evidence that Gb3 accumulation is the primary cause of Fabry disease.17 Here, A4GALT is linked to Fabry disease.