Massimo Zeviani

Sequestration of the polyunsaturated fatty acids protects the cells with oxidative phosphorylation deficiency from ferroptosis

The paper identifies that OXPHOS-deficient cells sequester polyunsaturated fatty acids into triacylglycerols to protect against ferroptosis, observed in patient fibroblasts and hypoxia conditions.

COA8 is a COX10-binding protein involved in the early biogenesis of cytochrome c oxidase

This study identifies COA8 as a COX10-binding protein essential for COX10 stability and catalytic activity in human cytochrome c oxidase biogenesis.

Study of an FBXO7 patient mutation reveals Fbxo7 and PI31 co-regulate proteasomes and mitochondria

This study identifies an FBXO7 mutation that disrupts its interaction with PI31, affecting proteasome and mitochondrial function in patient cells.

In vivo and in vitro mechanistic characterization of a clinically relevant PolγA mutation

This study characterizes the molecular pathogenesis of a POLG mutation by showing how it impairs POLγA function and leads to mtDNA instability through LONP1-dependent degradation.

Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer's disease-like pathology in human cerebral organoids

This study shows that PITRM1 deficiency in human cerebral organoids leads to Alzheimer's-like pathology, including protein aggregates and tau pathology, while 2D cultures do not show these features.

Neural stem cells traffic functional mitochondria via extracellular vesicles to correct mitochondrial dysfunction in target cells

This study shows neural stem cells transfer functional mitochondria via extracellular vesicles to restore mitochondrial function in target cells, improving disease outcomes in multiple sclerosis.