HCEMM-SU Neurobiology and Neurodegenerative Diseases Research Group

Running Projects

MODEL iN: Modelling age-related diseases using induced neurons directly reprogrammed from patient’s fibroblasts

Neurodegenerative disorders are a growing burden in our aging society. With longer life expectancy neurodegenerative disorders will become an even more serious public health issue influencing the active time of the elderly. Accumulating evidence are pointing at impairments in autophagy, a lysosomal degradation pathway, as playing a critical role in ageing and age-related neurodegenerative diseases. However, a clear mechanistic understanding of how alterations in autophagy causes cellular dysfunction and neuronal death, is currently lacking.
The overall aim of this project is to understand how alterations in autophagy contribute to the pathophysiology of chronic incurable neurodegenerative disorders of the central nervous system, such as Alzheimer’s Disease (AD), Huntington’s disease (HD) and Parkinson’s disease (PD). More specifically the aims of this study is to:
i) shed light on impairments of autophagy in neurodegenerative diseases of the CNS,
ii) screen for drugs restoring these defects,
iii) validate the best candidates.
This will be achieved by using models that we have recently developed specifically to study autophagy impairments in neurodegenerative diseases. The model is based on direct neuronal reprogramming of patient derived fibroblasts. This approach will allow to tackle the vulnerability of neurons to different protein build up in a system that is not only of human origin, but also patient specific with carrying the aging signature of the donor.
This information will be instrumental to develop novel treatment strategies for proteinopathies, including Parkinson’s, Huntington’s and Alzheimer’s disease, given their shared alteration in autophagy.
This project is supported by: HCEMM, STIA-FIKP

AGEiN: Neuronal autophagy in human ageing

Ageing affects all humans and is the most critical parameter for quality and length of life. Ageing is also by far the most critical risk factor for a majority of neurodegenerative disorders, including Alzheimer’s disease. Accumulating pieces of evidence point at impairments in autophagy, a lysosomal degradation pathway, as playing a critical role in ageing and age-related neurodegenerative diseases. However, a clear mechanistic understanding of how autophagy declines with age and causes cellular dysfunction and neuronal death, is currently lacking due to shortcomings of human neuronal models that recapitulate the ageing signatures of the donor.
The overarching objective of the proposed project is to define how neuronal autophagy declines during physiological healthy ageing in humans.
This project is supported by: HCEMM, STIA-FIKP

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