As we mark #MitochondrialDiseaseAwarenessWeek2026 (14-20 September), I would like to recognise the extraordinary global community of patients, families, clinicians, researchers, healthcare professiona...

Kücükköse, Luzarowski and colleagues show that removing a single N-terminal amino acid during the maturation of newly imported mitochondrial proteins globally stabilizes mitochondrial protein complexe...

Taskin, Shankar, Peselj, et al. demonstrate that mitochondria possess an intrinsic surveillance system that senses mild oxidative stress and activates the UPRmt before damage signals reach the cytosol...

Correct protein stoichiometry is essential for cellular function, and mitochondrial oxidative phosphorylation presents a particular challenge that requires tight control of the electron transport chain (ETC) and ATP synthase ratio. Moretti-Horten et al. identify the MiRA platform on which ETC and ATP synthase biogenesis are synchronized by a stop-and-go mechanism.

Most mitochondrial proteins are imported from the cytosol via the TOM complex. Here, Marada et al. identify DYRK1A as a surveillance kinase that synchronizes the different import routes to shape the o...