Tatyana A. Shelkovnikova

This study shows that the NTD of TDP-43 binds UG-rich RNA on monomers, while oligomerization is essential for repressing cryptic exons.

This study identifies TDP-43-independent mechanisms of STMN2 depletion in ALS, showing acute stress causes proteasomal degradation and translational repression, while chronic stress leads to reduced protein levels, with mRNA upregulation as a compensatory mechanism in some subtypes.

This study shows that senescent human cells secrete misfolded proteins via the endo-lysosomal system, driven by DNAJC5 and reduced intracellular proteostasis.

This study shows that FUS RNA condensation regulates its splicing and that disrupting this process can reduce FUS levels, offering a potential therapeutic strategy for ALS-FUS.

This study uses optogenetics to model C9orf72 poly-PR condensation in cells, revealing anisotropic nuclear condensates that cause TDP-43 pathology without stress response activation.

The paper identifies CCDC146 as a modifier of ALS survival through primary cilia dysfunction and shows ASO targeting of CCDC146 rescues ALS phenotypes in human and mouse models.

This study identifies a novel RNA-binding function of PGC-1alpha that couples transcriptional co-activation with mRNA nuclear export and mitochondrial homeostasis.

This study shows that CLN3 deficiency disrupts stress granule dynamics and translation, linking it to neurodegenerative disease mechanisms.

This study shows that NEAT1 regulates adaptive behavioural responses to stress in mice by affecting neuronal function and gene expression.

The paper shows that CREST toxicity in yeast and flies is reduced by PBP1/ATXN2 deletion, suggesting ATXN2 as a therapeutic target for ALS.