Abstract
Mutations in mitochondrial DNA (mtDNA) or depletion of the mitochondrial genome have been linked to several human diseases including Diabetes, heart disease, Parkinson's, Alzheimer's and cancer.1 The mitochondrial genome encodes 13 subunits of the respiratory complexes, the mitochondrial rRNAs and some tRNAs.1 Surprisingly though, it is possible to generate viable cell lines completely lacking in mitochondrial DNA termed Rho-0 cells.2 Recently, several groups have taken advantage of this fact to analyze the proteome of these cells to better understand how alterations in the mitochondrial genome can contribute to disease.