Mitochondria are organelles involved in a variety of cellular functions that are central to the life and death of a cell. Oxidative phosphorylation (OXPHOS), the main energy provider of the cell, takes place inside mitochondria and is known to be altered in carcinogenesis and tumor progression contributing to the “metabolic reprogrammingâ€, one of the hallmarks of cancer cells. Due to the central role of energy metabolism in cancer cell pathogenesis, mutations in the mitochondrial genome (mtDNA), which encodes for essential components of the OXPHOS pathway, have been suggested to play a role in many cancers, including prostate cancer. Recent studies provide evidence for increased levels of mutant mtDNA in prostate cancer patients with higher Gleason grade and relapse, as well as in bone metastatic sites. In this review, we will provide an overview of recent studies investigating the presence of mtDNA mutations in prostate cancer cells and their significance in the context of clinical pathological features of prostate cancer
