University of Toronto
The TAR DNA-Binding Protein (TDP-43) and Amyotrophic Lateral Sclerosis
Motor neurone diseases
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two devastating neurodegenerative diseases that share a number of commonalities. One major commonality is the presence of abnormal accumulations of a protein called TDP-43 in the brain and spinal cord of affected individuals. We are trying to understand how these abnormalities of TDP-43 occur and how they contribute to the disease process. We have already made major strides through the development of new disease models and use of cutting edge technologies to uncover an important disease pathway that links not only ALS and FTD, but also Alzheimer’s and Parkinson’s disease. Through further investigation we hope to establish this link with the aim of gaining a fuller understanding of the mechanistic basis of how TDP-43 is related to disease causation. It is through this that we hope to develop effective treatments.