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Aggregation of the RNA-binding protein TDP-43 is the key neuropathological feature of neurodegenerative diseases, including ALS and FTLD. TDP-43 is a ubiquitously expressed, nucleic acid-binding protein essential for human development. In physiological conditions, TDP-43 is predominantly nuclear, fo...
ORGANISM(S): Homo sapiens (Human) 
2023-08-31 | PXD041795 | Pride
Neurodegeneration in ALS and FTD results from both gain of toxicity and loss of normal function of the RNA-binding protein TDP-43, but their mechanistic connection remains unclear. Increasing evidence suggests that TDP-43 aggregates act as self-templating seeds, propagating pathology through the cen...
ORGANISM(S): Homo sapiens (Human) 
2025-04-14 | PXD058981 | Pride
Mutations disrupting the nuclear localization of the RNA-binding protein FUS characterize a subset of amyotrophic lateral sclerosis patients (ALS-FUS). FUS regulates nuclear RNAs, but its role at the synapse is poorly understood. Using super-resolution imaging we determined that the localization of ...
ORGANISM(S): Mus musculus (Mouse) 
2021-05-05 | PXD024075 | Pride
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