Understanding the Role of the Immune System in Frontotemporal Dementia

Principal Investigator

Project Goals

This project aims to understand the role of the peripheral immune system and lysosomal dysfunction in Frontotemporal Dementia FTD. Specific aim 1: Determine the requirement of infiltrating immune cells into the brain in FTD. Using mouse models of FTD, I will determine if dysfunctional immune cells in the periphery are responsible for the neurodegeneration seen in FTD, and if this can be reversed by replacing these with healthy immune cells. Specific aim 2: Assess immune and lysosome dysfunction in immune cells from FTD patients and determine role of the GPNMB protein in this. Lysosomes are cellular organelles that play a role in regulating the immune response. Progranulin, a protein implicated in FTD, regulates lysosomes.

Project Summary

The completion of proposed studies will provide a clearer understanding of the mechanisms underlying the role of the peripheral immune system in FTD and will inform rational design of new therapies. Indeed, if the peripheral immune system is instrumental in the aetiology of FTD, it may represent viable therapeutic targets that are in the periphery and therefore more easily accessible than those in the brain, thereby changing the landscape to improve future therapies for patients.If the peripheral immune system is instrumental in the aetiology of FTD, it may represent viable therapeutic targets that are in the periphery and therefore more easily accessible than those in the brain, thereby changing the landscape to improve future therapies for patients.

First published on: November 08, 2021

Last modified on: April 28, 2024