Fig. 2.

Changes of mitochondrial TMRM fluorescence induced by oligomycin in muscle cells isolated from healthy donors and from UCMD and Bethlem myopathy patients before and after treatment with CsA. Muscle cells obtained from biopsies of two healthy donors (a and b), four patients with UCMD (c–f; patients 1–4, respectively), and one with Bethlem myopathy (g; patient 5) were seeded onto glass coverslips precoated with laminin/poly-l-lysine, loaded with TMRM and studied as described in Experimental Procedures. The procedure was repeated after 1 month of oral treatment with 5 mg/kg CsA per day (c′–g′; patients 1–5, respectively). Where indicated, 6 μM oligomycin (arrows) or 4 μM carbonylcyanide-p-trifluoromethoxyphenyl hydrazone (arrowheads) was added. Each line represents one individual cell.