CORDIS Project
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This project focuses on understanding motor neuron disease by investigating the role of TDP-43 protein aggregation, which is prevalent in over 90% of patients. The goal is to identify compounds that can reduce TDP-43 aggregation and toxicity, potentially leading to new treatments for motor neuron disease and fronto-tem…
Until recently, the majority of motor neuron disease (MND) research has used cellular and animal models of disease based on the over-expression of mutant superoxide dismutase (SOD1). SOD1 mutations cause protein aggregation and toxicity but are found in only 5% of all MND patients.
In contrast, aggregates of the TAR DNA binding protein (TDP-43), have recently been identified in motor neurons and glia in greater than 90% of MND patients, and in cortical neurons in 60% of fronto-temporal lobar dem…
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