The Cascade of Consequences in Huntington Disease From Mutant Httex1 Synthesis and Aggregation

Grant number: 1184166 | Funding period: 2020 - 2022

Completed

Abstract

Huntington’s Disease is caused by the abnormal clustering of a mutated protein in neurons, which is hypothesized to trigger pathogenesis. The goal of this project is to determine how clustering does this and in particular what the impact is of clustering on the functioning of neurons in a mouse model of Huntington Disease. The research is aimed at advancing knowledge of the molecular cause of disease, which is needed in the design of therapeutic strategies to combat the dysfunctional effects.

Related publications (3)

Longitudinal spatial mapping of lipid metabolites reveals pre-symptomatic changes in the hippocampi of Huntington's disease transgenic mice

F Farzana, M McConville, T Renoir, S Li, S Nie, H Tran, AJ Hannan, DM Hatters, BA Boughton

2023-01-01

In Huntington's disease (HD), a key pathological feature includes the development of inclusion-bodies of fragments of the mutant h..

Flipping the switch: How cysteine oxidation directs tau amyloid conformations

DM Hatters

2021-11-01

Tau can adopt distinct fibril conformations in different human neurodegenerative diseases, which may invoke distinct pathological ..

Nascent mutant Huntingtin exon 1 chains do not stall on ribosomes during translation but aggregates do recruit machinery involved in ribosome quality control and RNA

AR Ormsby, D Cox, J Daly, D Priest, E Hinde, DM Hatters

2020-07-01

Mutations that cause Huntington's Disease involve a polyglutamine (polyQ) sequence expansion beyond 35 repeats in exon 1 of Huntin..

University of Melbourne Researchers

Danny M Hatters