Jonas Mechtersheimer

This study uses single-cell RNA sequencing to identify shared mitochondrial dysfunction in motor neurons across FUS and TARDBP ALS mutations, suggesting common toxic mechanisms.

The paper describes the generation and validation of C9orf72 repeat expansion knock-in iPSC lines for ALS and FTD research, showing they differentiate into neurons and display disease-related pathologies.

The paper develops a method to purify phase-separated FUS droplets and shows that LLPS alters FUS interactome composition, linking it to chromatin remodeling and mitochondrial function in ALS.

This study shows that FUS-dependent liquid-liquid phase separation is essential for early DNA damage response activation and repair complex assembly.