Leucocyte glutathione peroxidase activity and selenium level in Batten's disease

The glutathione peroxidase (GSHPx) activity was found significantly reduced in whole lymphocytes from patients suffering from Batten's disease. By means of two different procedures for isolation of subcellular fractions of lymphocytes it was possible to demonstrate an increased GSHPx activity of the particulate fractions (20.000 g-av and 105.000 g-av precipitates). However, the GSHPx activity of the supernatants was decreased. The GSHPx activity of the 20.000 g-av supernatant correlated significantly with the serum selenium content both in normal controls and in Batten's disease. A similar correlation was traced with GSHPx activity of erythrocyte haemolysate and serum selenium level in Batten's disease but not in normal controls. The GSHPx activity was also found decreased in the 20.000 g-av and 105.000 g-av supernatants of granulocytes. The abnormal subcellular distribution of GSHPx activities was related to the distribution of enzymes which were used as markers for different subcellular components. The data presented are discussed in relationship to the theory that Batten's disease is due to an increased peroxidation damaging the cellular membranes.