Fig. 4

Altered expression of Kir4.1, AQP4 and glutamine synthetase in Alpers’ syndrome patient astrocytes. (a) Immunofluorescence to identify Kir4.1 (purple), (b) AQP4 (orange) and (c) glutamine synthetase (red), using GFAP+ (green) and Hoescht (blue, nuclear marker) to identify individual reactive astrocytes in the occipital cortex. Scale bars = 10 μm. The mean optical intensity of Kir4.1, AQP4 and glutamine synthetase within individual GFAP + astrocytes are presented as circles. For Pt.11 – Pt.13 occipital cortex data, orange circles denote astrocytes imaged in lesioned cortex, and red circles denote astrocytes imaged in adjacent non-lesioned cortex. Box plots demonstrate the median, upper/lower quartiles and range of data. Multiple comparison analyses relative to control data: *P < 0.05, ** P < 0.01, *** P < 0.001, **** P < 0.0001. Note: Kir4.1 intensity was selectively increased in astrocytes from lesioned cortex of Pt.13 (P < 0.0001). N = 5 controls, N = 5 SUDEP