Fig. 1

Reactive astrogliosis in Alpers’ syndrome. (a) Increased intensity and distribution of glial fibrillary acidic protein (GFAP) + labelling in occipital cortex tissues from patients with Alpers’ syndrome (Pt.11 and Pt.13) relative to controls and SUDEP patients. GFAP + labelling is intense in the necrotic lesion of Pt.11 (red arrow head), and shows selective cortical layer involvement in adjacent preserved tissue (blue arrow head). Hypertrophic ameboid GFAP + astrocytes are abundant across all cortical layers of Pt.13. Scale bars = 100 μm. (b) Quantitative analysis of the percentage area of cortical GFAP + staining revealed significantly increased GFAP + labelling in multiple Alpers’ syndrome patient tissues compared to controls (N = 8) [40]. Data presented as mean + SD. Multiple comparison analyses relative to control data: ** P < 0.01, *** P < 0.001. (c) Analyses assessing the correlation between the percentage area of GFAP + labelling with the density of interneuron subtypes (parvalbumin+, calretinin+, calbindin + and somatostatin+) and pyramidal neurons (SMI-32+), expressed as z-scores, using our published neuronal density data set [19]. Legend = Spearman-rank r value. * P < 0.05, ** P < 0.01