Figure 2.
35S-methionine pulse labelling of myoblast (A and B) and fibroblast (C) cell lines. Patients (P) 1, 19 and 36 showed a significant overall reduction in mitochondrial oxidative phosphorylation complex subunits. Patients 32 and 16 showed an isolated decrease in the translation of ND4. Patient 12 had reduced steady-state levels of ND1, ND4 and ND5, with normal ND2, and COXI and ND4 were decreased in Patient 34. Patient 9 contained a structurally altered, smaller COXI mitochondrial protein. Deficient bands and lanes are marked with white arrows and stars.