Fabry disease is an X-linked condition due to deficiency of the lysosomal enzymeα- galactosidase-A leading to accumulation of globotriaosylceramice (GL-3), the main substrate of the deficient enzyme, in various cell types. Fabry nephropathy is one of the major complications of Fabry disease. We have shown that podocyte accumulation of GL-3 and podocyte injury are progressive with age in young patients. We have also shown that patients with Fabry disease lose podocytes in the urine. Novel methods developed in our lab to quantify GL-3 inclusion volume in podocytes, for the first time, documented partial clearance of podocytes from GL-3 in male adult patients following one year of enzyme replacement therapy. Najafian Lab continues to work on studying Fabry disease complications by developing new methods for assessment of cellular injury and GL-3 accumulation, as well as in-vitro models of the disease that can help us better understand pathophysiology of this condition.