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Fig. 6 | Orphanet Journal of Rare Diseases

Fig. 6

From: Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model

Fig. 6

Local astroglial activation in the barrel field of primary somatosensory cortex of the homozygous Bcs1l c.232A>G mouse. (a) Immunostaining for the astroglial marker glial fibrillary acidic protein (GFAP) reveals localized astroglial activation in the barrel field of the primary somatosensory cortex (S1BF) of Bcs1l c.232A>G mouse, but not in control animals. (b) Schematic representation of the S1BF according to Paxinos and Franklin (2001) [36]. (c) Astroglial activation shows a pattern in which the upper part of cortical layer V is less affected (g, b for ctrl) compared to the more strongly affected layers II-IV (f, a for ctrl) and lower part of layer V and upper part of layer VI (h, c for ctrl). The areas in the insets a-c and f-h are shown in figures d and e, respectively. (d) No differences in the amount or the phenotype of the microglial cells were seen in the Bcs1l c.232A>G mouse compared to the control. Scale bars (a) 500 μm, (b) d and e 100 μm and a-c, f-h 50 μm, (c) 100 μm

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