In rare, hereditary storage diseases such as Sandhoff’s disease or Tay-Sachs syndrome, the metabolic waste from accumulating gangliosides cannot be properly disposed of in the nerve cells because important enzymes are missing. The consequences are grave: They range from movement restrictions to blindness, mental decline and early death. Scientists at the University of Bonn now demonstrate why these gangliosides also accumulate in patients with other storage diseases and cause a deterioration in them.
Source: EurekaAlert, https://www.eurekalert.org