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Distinct responses of neurons and astrocytes to TDP-43 proteinopathy in ALS
Scientists from UK and Taiwan have developed a modeling platform at the cellular level, which summarizes the key aspects of sporadic ALS and demonstrates both an initial neuroprotective role for astrocytes and the cell-type specific toxic effect of TDP-43 oligomers.
In addition, these scientists demonstrated that highly purified recombinant TDP-43 oligomers can reproduce this specific cell-type toxicity, providing additional support for a hypothesis of mediated toxicity. by protein oligomers in ALS, i.e. prion-like behavior.
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