TDP-43 proteinopathy

TDP-43 proteinopathy involves abnormal changes in the TDP-43 protein within nerve cells. Under normal conditions, TDP-43 helps regulate gene expression and cell health. In certain neurodegenerative diseases like ALS and some forms of frontotemporal dementia, TDP-43 misfolds and accumulates into abnormal clumps. These clumps impair cell function and can lead to cell death, contributing to the progressive brain and nerve damage seen in these diseases. Essentially, TDP-43 proteinopathy reflects a malfunction in how this protein is processed, leading to harmful buildup that disrupts neural integrity.