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MRC Mitochondrial Biology Unit


In a collaborative research article published today in Nature Communications, Pedro Rebelo-Guiomar and MBU colleagues from the research groups of Drs Michal Minczuk and Alex Whitworth at the MBU report the identification of a key checkpoint during mtLSU assembly, which is essential to maintaining mitochondrial homeostasis.

Many cellular processes, including ribosome biogenesis, are regulated through post-transcriptional modification of RNA. This work describes how the protein MRM2 controls the release of energy (mitochondrial respiration) by regulating the generation of new mitochondrial ribosomes (mitoribosome biogenesis). The authors found that disruption of the MRM2 Drosophila melanogaster orthologue leads to mitochondria-related developmental arrest, providing a mechanistic explanation for mitochondrial disorders associated with this protein in humans and providing a tool for future investigation of mitochondrial dysfunction and their tissue-specificity.

This research study was led by Dr Michal Minczuk and involved collaborators at the University of Cambridge, The Weizmann Institute of Science (Israel), and STORM Therapeutics (Cambridge).

Full publication reference:

Rebelo-Guiomar, P., Pellegrino, S., Dent, K.C. et al. A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit. Nat Commun 13, 929 (2022).