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DYSREGULATION OF THE (IMMUNO)PROTEASOME PATHWAY IN MCD
Figure 1 β1 proteasome subunit immunoreactivity in control, FCD type IIa, FCD type IIb and TSC. Panels A and B: (control) show weak immunoreactivity (IR) in both cortex (A; insert: neuron with weak expression of β1 subunit) and white matter (B; not detectable glial expression). Panel C (FCD IIa) shows positive dysmorphic neurons (arrows; insert: high magnification) and glial cells (arrow-heads). Panels D-E (FCD IIb) show several β1 positive cells within cortex (D) and white matter (E), including dysmorphic neurons (arrows in D), glial cells (arrow-heads and insert in D) and balloon cells (arrows in E; prominent nuclear expression; insert: high magnification). Panels F-G (TSC-tuber): β1 subunit expression is observed within the tuber in dysmorphic neurons (ar- rows in F; insert a: co-localization with the neuronal marker NeuN; insert b, co-localization with GFAP), glial cells (arrow-heads in F) and in giant cells (asterisk in G; insert: co-localization with GFAP). additional examples of the different cells types at higher magnification in separate speci- mens. The inserts within panels show imagines of the different cells types at higher magnification in separate specimens. Scale bar in G: A-C, E-F: 80 μm; D-G: 40 μm.
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