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Related Experiment Video

Updated: May 7, 2026

Sensitive Detection of Proteopathic Seeding Activity with FRET Flow Cytometry

Sensitive Detection of Proteopathic Seeding Activity with FRET Flow Cytometry

Published on: December 8, 2015

Neurodegenerative lesions: seeding and spreading.

¹Laboratoire de neuropathologie Raymond-Escourolle, hôpital de la Pitié-Salpêtrière, 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France; Centre de recherche de l'ICM, équipe Alzheimer-Prion, 47, boulevard de l'Hôpital, 750713 Paris, France.

Revue Neurologique

|September 17, 2013

Summary

Neurodegenerative diseases involve protein accumulation, not just neuron loss. Misfolded proteins spread like prions, causing disease progression via neuronal connections.

Keywords:

Alzheimer Alzheimer disease Argyrophilic grain Argyrophilic grains disease Astrocytic plaque Corps de Lewy Cortico-basal degeneration Dégénérescence cortico-basale Dégénérescences neurofibrillaires Grain argyrophile Lewy body Maladie des grains argyrophiles Neurofibrillary tangle Paralysie supranucléaire progressive Parkinson Parkinson disease Plaque astrocytaire Plaques séniles Prion Prion-like Progressive supranuclear palsy Seeding Senile plaque Touffe astrocytaire Tufted astrocyte

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Area of Science:

Neurobiology
Neuropathology
Molecular Biology

Background:

Neurodegenerative diseases are increasingly defined by abnormal protein accumulation rather than solely neuronal loss.
While mutations can alter protein structure, most disease-associated proteins have a normal primary sequence.
Pathological hallmarks in Alzheimer's, Parkinson's, and progressive supranuclear palsy suggest disease spread follows neuroanatomical pathways.

Purpose of the Study:

To investigate the prion-like mechanisms of protein misfolding and aggregation in neurodegenerative diseases.
To explore whether exogenously introduced protein aggregates can initiate disease pathology in experimental models.
To understand the cellular mechanisms, such as endocytosis and transport, involved in the propagation of misfolded proteins.

Main Methods:

In vivo studies involving the injection of protein aggregates (Aβ, tau, alpha-synuclein) into the brains of transgenic and wild-type mice.
In vitro cell culture experiments to assess protein uptake and transport by neurons.
Analysis of neuropathological hallmarks in experimental models to mimic human disease progression.

Main Results:

Brain homogenates with amyloid-beta (Aβ) aggregates induced Aβ deposition in APP transgenic mice.
Tau aggregates from human tauopathies induced tau pathology in mice expressing normal human tau.
Synthetic alpha-synuclein fibrils initiated Parkinson's-like pathology in wild-type mice.
Cell culture data indicated that specific oligomeric or fibrillar states are necessary for protein endocytosis and neuronal transport.

Conclusions:

The protein accumulating in neurodegenerative diseases is initially misfolded.
This misfolded protein can contaminate normal proteins in a prion-like manner, driving disease progression.
Neuronal connections play a critical role in the cell-to-cell transmission of these misfolded proteins.