GLUTATHIONE LEVELS AND NERVE-CELL LOSS IN HIPPOCAMPAL CULTURES FROM TRISOMY-16 MOUSE - A MODEL OF DOWN-SYNDROME

The tripeptide glutathione (reduced state, GSH) is an important intrac ellular free radical scavenger protecting cells against oxidative stre ss. The trisomy 16 mouse is a model of the human trisomy 21 (Down synd rome). Here we demonstrate that cultured hippocampal neurons from tris omy 16 mouse exhibit decreased GSH levels and augmented cell death whe n compared to diploid cells. Additional lowering of GSH levels led to enhanced cell death in trisomy 16 cells. Based on these results we sug gest that a GSH level which is decreased under a specific threshold by increased consumption, I:educed synthesis or lack in precursor contri butes to cell loss and neurodegeneration in Down syndrome. (C) 1997 El sevier Science B.V.