Complex I Complex III Complex IV Complex II Supplemental Figure 3

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Complex I Complex III Complex IV Complex II Supplemental Figure 3 Blue native polyacrylamide gel electrophoresis analysis of skin fibroblasts. Twenty µg proteins of isolated mitochondria from fibroblasts were solubilized in dodecyl maltoside and subjected to blue native polyacrylamide gel electrophoresis and Western blotting. The amount of fully assembled complex IV was shown to be slightly decreased both in this patient and another one with L122V. Control1 Patient L122V Control2 Complex I kDa 669 - 440 - 232 - 134 - Complex III Complex IV Complex II