Cyclosporine A in Ullrich Congenital Muscular Dystrophy: Long-Term Results

Figure 1

Changes of mitochondrial TMRM fluorescence induced by oligomycin in muscle cells isolated from patients before and after treatment with CsA. Muscle cells obtained from biopsies of two patients (P5 in a and a′, P6 in b and b′) were seeded onto glass coverslips precoated with laminin/poly-L-lysine, loaded with TMRM, and studied as described in Section 2. The procedure was repeated before (a, b) and after (a′, b′) 3 months of oral treatment with CsA. Where indicated, 6 μM oligomycin (Oligo) and 4 μM carbonylcyanide-p-trifluoromethoxyphenyl hydrazone (FCCP) were added. Each line represents one individual cell. Depolarized cells were 59% before (a) and 13% after (a′) CsA treatment in Patient 5, and 69% before (b) and 15% after (b′) CsA treatment in Patient 6.