Molecular pathology of neurodegenerative diseases by cryo-EM of amyloids

SHW Scheres, B Ryskeldi-Falcon, M Goedert - Nature, 2023 - nature.com
Abnormal assembly of tau, α-synuclein, TDP-43 and amyloid-β proteins into amyloid
filaments defines most human neurodegenerative diseases. Genetics provides a direct link …

Emerging trends in cryo-EM-based structural studies of neuropathological amyloids

S Mishra - Journal of Molecular Biology, 2023 - Elsevier
Tauopathies, synucleinopathies, Aβ amyloidosis, TDP-43 proteinopathies, and prion
diseases-these neurodegenerative diseases have in common the formation of amyloid …

Mouse α-synuclein fibrils are structurally and functionally distinct from human fibrils associated with Lewy body diseases

A Sokratian, Y Zhou, M Tatli, KJ Burbidge, E Xu… - Science …, 2024 - science.org
The intricate process of α-synuclein aggregation and fibrillization holds pivotal roles in
Parkinson's disease (PD) and multiple system atrophy (MSA). While mouse α-synuclein can …

Saturation mutagenesis of α-synuclein reveals monomer fold that modulates aggregation

J Chlebowicz, W Russ, D Chen, A Vega, S Vernino… - Science …, 2023 - science.org
α-Synuclein (aSyn) aggregation underlies neurodegenerative synucleinopathies. aSyn
seeds are proposed to replicate and propagate neuronal pathology like prions. Seeding of …

[HTML][HTML] Deep learning-based structure modelling illuminates structure and function in uncharted regions of β-solenoid fold space

S Mesdaghi, RM Price, J Madine, DJ Rigden - Journal of Structural Biology, 2023 - Elsevier
Repeat proteins are common in all domains of life and exhibit a wide range of functions. One
class of repeat protein contains solenoid folds where the repeating unit consists of β-strands …

Multifaceted interactions mediated by intrinsically disordered regions play key roles in alpha synuclein aggregation

SD Khare, P Chinchilla, J Baum - Current opinion in structural biology, 2023 - Elsevier
Abstract The aggregation of Alpha Synuclein (α-Syn) into fibrils is associated with the
pathology of several neurodegenerative diseases. Pathologic aggregates of α-Syn adopt …

On the pH-dependence of α-synuclein amyloid polymorphism and the role of secondary nucleation in seed-based amyloid propagation

L Frey, D Ghosh, BM Qureshi, D Rhyner… - Elife, 2024 - elifesciences.org
The aggregation of the protein α-synuclein is closely associated with several
neurodegenerative disorders and as such the structures of the amyloid fibril aggregates …

Role of conformational dynamics in pathogenic protein aggregation

X Sun, HJ Dyson, PE Wright - Current opinion in chemical biology, 2023 - Elsevier
The accumulation of pathogenic protein oligomers and aggregates is associated with
several devastating amyloid diseases. As protein aggregation is a multi-step nucleation …

α-Synuclein Strains and Their Relevance to Parkinson's Disease, Multiple System Atrophy, and Dementia with Lewy Bodies

NJ Graves, Y Gambin, E Sierecki - International Journal of Molecular …, 2023 - mdpi.com
Like many neurodegenerative diseases, Parkinson's disease (PD) is characterized by the
formation of proteinaceous aggregates in brain cells. In PD, those proteinaceous aggregates …

Dopaminergic system and neurons: role in multiple neurological diseases

H Chen, J Li, Z Huang, X Fan, X Wang, X Chen… - …, 2024 - Elsevier
The dopaminergic system is a complex and powerful neurotransmitter system in the brain. It
plays an important regulatory role in motivation, reward, cognition, and motor control. In …