Stem cells require tight control of energy metabolism to maintain homeostasis. They possess few immature mitochondria, repress mitochondrial respiration and instead use glycolysis to produce energy, yet mitochondrial defects can lead to severe stem cell dysfunction. Recent studies have shown that mitochondrial mass, function and integrity are tightly controlled in stem cells and the integrity of the mitochondrial genome is equally important to nuclear genome integrity for proper stem cell homeostasis. Mitochondria are now considered central in regulating stem cell function and governing cellular fate choices. This review will summarize recent advances highlighting the importance of mitochondrial integrity in stem cells.
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