Hu. Shetty et al., Ts65Dn mouse, a Down syndrome model, exhibits elevated myo-inositol in selected brain regions and peripheral tissues, NEUROCHEM R, 25(4), 2000, pp. 431-435
myo-Inositol is elevated in the Down syndrome (DS; trisomy 21) brain and ma
y play a role in mental retardation. In the present study, we examined brai
n regions and peripheral tissues of Ts65Dn mouse, a recently characterized
genetic model of DS, for abnormal myo-inositol accumulation. A GC/MS techni
que was used to quantitate myo-inositol and other polyol species (ribitol,
arabitol, xylitol, and 1,5-anhydrosorbitol) in tissues from the Ts65Dn mice
and control diploid mice. myo-Inositol was found to be elevated in frontal
cortex, hippocampus, and brain stem but not in cerebellum of the Ts65Dn mo
use. Among peripheral organs examined, liver and skeletal muscle were found
to excessively accumulate myo-inositol. In all tissues, concentrations of
polyol internal controls were normal. The Ts65Dn mouse is useful to study t
he possible effect of elevated myo-inositol on cellular processes.