Figure 5.

Immunofluorescence studies on VCP–mutant and control fibroblasts at basal conditions and after arimoclomol (ari) treatment. Arimoclomol treatment resulted in cytoplasmic increase of FYCO1, which is more pronounced in controls (control 1 is shown as one representative control). Decrease of p62–immunoreactivity within the nuclei of control fibroblasts treated with arimoclomol and p62 enrichment within the cytoplasm of VCP–mutant fibroblasts. CD63 showed an increased cytoplasmic immunoreactivity in both control and patient derived fibroblasts after arimoclomol treatment. A similar finding was obtained for tenascin. Scale bars = 50 µm. Two representative patients are shown: VCP fibroblasts 2 = patient 2 and VCP fibroblasts 3 = patient 3; patient numbering is consistent with patient numbering in Figure 1.