Fig. 3.

Changes of mitochondrial TMRM fluorescence induced by oligomycin in muscle cell cultures from a healthy donor and UCMD patients. Myoblasts from two healthy donors (A and A′) and from UCMD patients 1 (B–D) and 2 (B′–D′) were loaded with TMRM and studied as described in Materials and Methods. Cells were seeded on glass (A–C and A′–C′) or on collagen VI (D and D′). Where indicated by arrows, 6 μM oligomycin (O) and 4 μM carbonyl cyanide p-trifluoromethoxyphenylhydrazone (FCCP) (F) were added in the absence of further treatments (open symbols) or after treatment for 30 min with 1.6 μM CsA or 5 μM 1,2-bis(2-aminophenoxy)ethane-N,N,N′,N′-tetraacetic acid tetraacetoxymethyl ester (filled symbols in B and B′ and C and C′, respectively). Similar results were obtained with cells from patients 3 and 4, and the results shown are representative of at least five repeats per patient.