Jik Nijssen

This study shows that redox surveillance in stress granules prevents TDP-43 aggregation by maintaining a controlled redox environment.

This study investigates the role of beta2-microglobulin and HLA in motor neuron vulnerability in ALS, finding dynamic regulation but not major disease modification.

This study shows that TDP-43 aggregation in stress granules requires up-concentration and oxidative stress, leading to intra-condensate demixing and pathological aggregates.

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 identifies lipoamide as a small molecule that dissolves stress granules and improves outcomes in ALS models by modulating redox-sensitive proteins.

This study investigates mesoscale organization of TRIM proteins, finding that TRIM8 truncations cause condensatopathy by disrupting condensation and signaling pathways.

The paper identifies gene expression patterns in motor neurons that predict ALS across species and SOD1 mutations, revealing differences in vulnerability and resilience.

This study uses LCM-seq to analyze transcriptional changes in SMA, identifying cell-type specific adaptations that explain resistance in ocular motor neurons.

This study generates oculomotor neurons from mouse stem cells and shows they are more resilient to ALS-like toxicity than spinal motor neurons.

Axon-seq identifies axon-specific transcripts and ALS-dysregulated genes in motor axons, revealing novel therapeutic targets for motor neuron disease.