CYTOPLASMIC ABNORMALITIES IN CULTURED CEREBELLAR NEURONS FROM THE TRISOMY-16 MOUSE

This study represents a first effort to characterize the growth and de velopment of murine trisomy 16 neurons using single-cell neuron cultur e techniques. Murine trisomy 16 is a model for the human Down syndrome , or trisomy 21. Both show similar nervous system abnormalities includ ing decreases in cerebellar size and in numbers of cerebellar neurons. Trisomy 16 cerebellar neurons cultured from 17-gestational day concep tuses grew less extensive neuritic arbors than normal neurons. Unlike controls, the individual neurites of the trisomic neurons were not cle arly distinguishable as axons or dendrites over the 10 day period that they were observed. The trisomic neurons were characterized by dimini shed levels of microtubules, abnormally shaped mitochondria, and the p resence of dense bundles of abnormal filaments that were not observed in any of the normal littermate neurons. (C) 1998 Elsevier Science B.V . All rights reserved.