CORDIS Project
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This project focuses on understanding the role of glial cells in motor neurone disease by utilizing human-derived astrocytes to investigate toxic pathways affecting motor neurons. It aims to identify genetic factors contributing to this toxicity and test potential treatments using a mouse model of the disease.
Motor neurone disease (MND) is a neurodegenerative disease, characterized by progressive loss of motor neurons (MN), leading to muscle atrophy and death.
Approximately 10% of cases are familial, and 20% of these have been linked to dominant mutations in Cu/Zn superoxide dismutase 1 (SOD1).
Transgenic mice carrying mutant SOD1 develop a neuromuscular disease similar to human MND.
For nearly a decade MND has been considered a disease selectively affecting the MN, but strong evidence has accumulate…
THE UNIVERSITY OF SHEFFIELD
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