Elena I. Rugarli

The paper shows OMA1 regulates mitochondrial protein import and OXPHOS biogenesis under stress by degrading DNAJC15, affecting protein translocation and organelle communication.

The paper investigates the pathogenic mutation m.10191T>C in mitochondrial DNA, showing it leads to reduced complex I function and NADH accumulation, contributing to Leigh syndrome.

This study identifies AFG3L2-mediated proteolysis as a mechanism that restricts mitochondrial biogenesis and gene expression under hypoxia by degrading key mitochondrial proteins.

This study shows CLUH is essential for maintaining mitochondrial function and translation in motoneuronal axons, preventing peripheral neuropathy in mice.

This study identifies ERLIN1/2 scaffolds that mediate TMUB1-RNF170 interactions, regulate cholesterol esterification, and maintain ER cholesterol levels to control Golgi morphology and the secretory pathway.

The paper shows that the mitochondrial proton gradient regulates the m-AAA protease AFG3L2 through TMBIM5, linking protein turnover to mitochondrial energy status and Ca2+ signaling.

This study identifies YME1L as a mitochondrial protease that regulates metabolic state transitions in adult neural stem cells, ensuring self-renewal and preventing premature differentiation.