Figure 3.

A blue native PAGE gel analysis with in gel activity staining of the respiratory chain components in the liver of a patient with DGUOK mtDNA depletion disease. C = control. P= patient. I, II, IV, V are the respective mitochondrial complexes. The added bands of lower molecular weight for complex V in the patient reflect incomplete assembly of complex V due to missing mtDNA encoded subunits. The intensities of the bands for complex I and complex IV are also reduced in the patient. This profile is seen in generalized defects of the synthesis of mitochondrial DNA encoded subunits such as in mtDNA depletion, or in defects of transcription or translation of mtDNA encoded subunits.