Fig. 4

Filament but not sarcomeric proteins in vacuoles and membrane attack complex may line the membrane. a. Fluorescence microscopy reveal that filamin C protein (FLNC) and desmin may accumulate in the vacuoles whereas the sarcomeric protein actin does not. Both laminin α2 (merosin) and dystrophin (LNα2 & DYS in green), used to demonstrate the sarcolemma clearly line the vacuoles as well and in the case of laminin α2 this, appear to accumulate in the vacuoles as well. DAPI (blue) demonstrates nuclei and the merged pictures show that sporadic vacuoles may contain nuclei. Several fibres demonstrate invaginations in the laminin α2 and dystrophin stains. b. Membrane attack complex complement C5b-9 deposits are seen in fibres with vacuoles, both at the sarcolemma, membrane invaginations and inside vacuoles. Bar is 50 µm