Protein misfolding and aggregation in neurodegenerative diseases
MIRCen
Axe 1 : Génomique, cellules humaines, reprogrammation et neuro-organoïdes
Biochemistry, cell culture, microscopy, proteomics, spectroscopy
The neurodegenerative diseases of Alzheimer's, Parkinson's or Huntington's are proteinopathies due to the aggregation of distinct proteins in the central nervous system. These aggregated proteins propagate and multiply in the brain, causing progressive and irreversible degeneration of neuronal cells.
We demonstrated that aggregates of the same protein can differ in their structures. These “polymorphs” are capable of differentially targeting distinct neuronal populations and multiplying therein while maintaining their physical and pathological characteristics. This could contribute to the clinical heterogeneity observed in different neurodegenerative diseases due to the aggregation of the same protein.
Our work aims to:
- Dissect the molecular events leading to protein aggregation and the spread of pathologic protein aggregates from cell to cell
- Characterize the structure of the different polymorphs produced in vitro and derived from patients and to elucidate their pathological properties
- Develop approaches to counter the aggregation and spread of these polymorphs
18 route du Panorama, 92260, Fontenay-aux-Roses
Team leader :
Ronald Melki
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Key words : #Parkinson #Alzheimer #Huntington #protéinopathies #protéostase #Parkinson #Alzheimer #Huntington #diseases #proteinopathies #proteostasis