Programmed re-modeling of the heat shock response underlies onset of ALS

Project: Research project

Project Details


The causal relationship between aging and ALS remains mysterious; however, age-related changes in the quality of cellular proteins has been put forward as a trigger for the onset of neurodegeneration. Interestingly, an imbalance in protein quality control occurs during aging and is exacerbated in multiple neurodegenerative disorders, including ALS. This leaves cells vulnerable to the deleterious effects of errors in protein biogenesis, expression of mutant proteins, and exposure to environmental stress. Despite these observations, relatively little is known regarding the relationship between the age-related decline in protein quality control and ALS onset. This work will reveal how aging influences protein quality control pathways and reveal the genetic switches that regulate this process. Furthermore, by manipulating these switches we will understand the impact of the aging process and protein quality control on disease progression in models of ALS. This will lead to the identification of new regulators of protein homeostasis and novel therapeutic targets for ALS. (155 words)
Effective start/end date8/1/137/31/15


  • ALS Association (2212)


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