Evidence for Aberrant Astrocyte Hemichannel Activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL)
Figure 5
CLN3Δex7/8 astrocytes display significant reductions in molecules critical for glutamate homeostasis.
Immunofluorescence staining for glutamine synthetase and GLAST was performed on tissue sections from the somatosensory cortex (S1C), visual cortex (VC), hippocampus (HPC), striatum (STR), and thalamus (TH) of wild type (WT) and CLN3Δex7/8 mice at postnatal day 90. (A) Representative images depicting glutamine synthetase and GLAST expression in the STR with nuclei depicted by DAPI staining (blue; magnification, 20X; inset 50×50 µm zoom). (B) Glutamine synthetase and (C) GLAST immunoreactivity was quantitated in each brain region (n = 3–4 mice per group; 6 images/region for each animal) with results reported in arbitrary units (a.u.). Significant differences in glutamine synthetase and GLAST staining between WT and CLN3Δex7/8 tissues are denoted by asterisks (*p<0.05; **p<0.01; ***p<0.001).