Additional file 2: Figure S2. of Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons

Astrocyte Cultures stained with Glutamine Synthetase. Since GFAP expression can be down-regulated by astrocytes in culture, we also immunostained a parallel series of primary cortical astrocyte cultures from P1–2 wild type (WT) and Cln3-deficient (Cln3 −/− ) mice with glutamine synthetase as an additional marker of astrocyte phenotype, after an additional 48 h in culture. DAPI was used to visualize all nuclei. Virtually all the DAPI stained cells (blue) were also immunoreactive for glutamine synthetase (red) in both WT and Cln3 −/− cultures, and this was quantified as being 99.71 ± 0.15% (WT) and 99.29 ± 0.21% (Cln3 −/− ) of the DAPI stained cells, respectively. Scale bar = 20 μm. (TIFF 2129 kb