Phase separation and protein aggregation in neurodegenerative diseases

Hortense de La Seiglière, Ænora Letourneur, François Ichas, Francesca De Giorgi
Biophysical Chemistry. 2026-11-01; 338: 107678
DOI: 10.1016/j.bpc.2026.107678


de La Seiglière H(1), Letourneur Æ(1), Ichas F(2), De Giorgi F(3).

Author information:
(1)SynTeam, Institut des maladies neurodégénératives, CNRS UMR5293, Univ.
Bordeaux, France.
(2)SynTeam, Institut des maladies neurodégénératives, CNRS UMR5293, Univ.
Bordeaux, France; Laboratory of Human Anatomy, DiSTeBA, University of Salento,
Lecce, Italy.
(3)SynTeam, Institut des maladies neurodégénératives, CNRS UMR5293, Univ.
Bordeaux, France. Electronic address: .

Neurodegenerative diseases such as Alzheimer’s, Parkinson’s, frontotemporal
dementia, and ALS are characterized by amyloid protein aggregation involving
intrinsically disordered proteins that are also capable of liquid-liquid phase
separation (LLPS). LLPS, known to drive the formation of dynamic membraneless
organelles essential for cellular functions, can play a role in limiting
fibrillation process or aberrantly transition into solid aggregates under
pathological conditions. Here we review how mutations, post-translational
modifications, and environmental factors can modulate LLPS of proteins like Tau,
TDP-43, FUS, and α-synuclein, potentially regulating amyloid aggregation. We
also examine the interplay of these proteins exploring how LLPS and condensate
maturation could impinge on the emergence of co-pathologies contributing to
disease progression. Finally we discuss emerging therapeutic strategies, aimed
at modulating phase separation dynamics.

Copyright © 2026 The Authors. Published by Elsevier B.V. All rights reserved.

DOI: 10.1016/j.bpc.2026.107678
PMID: 42418847

Conflict of interest statement: Declaration of competing interest The authors
declare that they have no known competing financial interests or personal
relationships that could have appeared to influence the work reported in this
paper.

Auteurs Bordeaux Neurocampus