Li_1983_Am.J.Hum.Genet_35_629

Normal human urine has been found to contain activator proteins that stimulate the enzymic hydrolysis of GM1 and GM2 gangliosides. These two activators were partially purified by Sephadex G-200 filtration and DEAE-Sephadex A-50 chromatography. The presence of these two activators was assayed by demonstrating the stimulation of the in vitro hydrolysis of GM1 and GM2 gangliosides. As little as 50 ml of urine is sufficient to detect the presence of these two activators. The crude activator preparation from normal urine was also found to stimulate the hydrolysis of galactosylceramide sulfate by arylsulfatase A.