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Farzadfard, A.

Publications and source records attributed to Farzadfard, A..

3 recordsLinked to original sources

Probing the energy landscape of α-Synuclein amyloid fibril formation by systematic K-to-Q mutagenesis

The aggregation of natively disordered -Synuclein (Syn) into amyloid fibrils is a hallmark of Parkinsons and other neurodegenerative diseases. Understanding Syns pathological role remains a major challenge due to its complex, context-dependent energy landscape characterized by conformational plasticity and fibril polymorphism. Here, we present a systematic mutational analysis as a quantitative probe of the Syn energy landscape, focusing on electrostatic contributions to key aggregation pathways. We engineered Syn variants with one to eight lysine-to-glutamine substitutions and analyzed their aggregation under controlled conditions to delineate their effects on nucleation, elongation, seed amplification, fibril stability, and fibril polymorphism. We find that Syn aggregation from a homogenous solution can be modelled well using global properties, including protein concentration, charge, and ionic strength. Microscopic pathways and the resulting fibril polymorphs are instead modulated by sequence-specific effects. We identify mutations of residues found in fibril cores as perturbations that significantly modify the Syn free energy landscape, creating pathways and energy minima not accessible to the WT under the same experimental conditions. In contrast, mutations outside of the fibril core affect the magnitude of the relevant energy barriers whilst overall maintaining a WT-like free energy landscape. Our work outlines a scalable, quantitative framework that increases the informational output of the mutational studies of Syn using conventional assays. The approach can be extended by incorporating additional mutational and functional data to deepen our understanding of Syns energy landscape and its role in health and disease.

biophysics↗

Divergent effects of pathological α-synuclein truncations and mutations on phase separation

Phase separated condensates can accelerate -synuclein (-Syn) amyloid fibril formation implicated in Parkinsons disease pathogenesis. The effects of pathological modifications, i.e., truncations and familial mutations on the thermodynamics, material properties, and the extent of amyloid aggregation within -Syn condensates remain elusive. Here, we quantitatively demonstrate that terminal truncations significantly alter -Syn phase separation, while familial mutations impart minimal effects. Spontaneous sol-gel phase transitions of the truncated -Syn variants could give rise to amyloid fibrils almost instantly within condensates, suggesting similarities between molecular interactions driving both processes. Extending our study to model coacervate and condensate systems where -Syn acts as a client, we find -Syn can dissolve coacervates and form Pickering clusters on condensate surfaces--regulating their size. Additionally, the C-terminal region of -Syn modulates nucleic acid sequestration within condensates. Together, our findings reveal diverse effects of -Syn modifications on phase separation, both in pathological and physiological contexts. GRAPHICAL ABSTRACT O_FIG O_LINKSMALLFIG WIDTH=200 HEIGHT=151 SRC="FIGDIR/small/624073v1_ufig1.gif" ALT="Figure 1"> View larger version (52K): org.highwire.dtl.DTLVardef@1760f9dorg.highwire.dtl.DTLVardef@1fb155forg.highwire.dtl.DTLVardef@138402dorg.highwire.dtl.DTLVardef@1fd5229_HPS_FORMAT_FIGEXP M_FIG C_FIG

biophysics↗

Specific inhibition of α-synuclein oligomer generation and toxicity by the chaperone domain Bri2 BRICHOS

Understanding the molecular mechanisms of neurodegenerative diseases and finding efficient treatments have been major priorities for research and society, yet new therapeutic approaches remain essential to face the socio-economic burden caused by these devastating diseases. Protein misfolding and aggregation are involved in several neurodegenerative disorders, such as -synuclein (Syn) implicated in Parkinsons disease. Elucidating the microscopic nucleation mechanisms has opened new opportunities to develop therapeutics against toxic mechanisms and species. Here, we show that naturally occurring molecular chaperones, represented by the anti-amyloid Bri2 BRICHOS domain, can be used to target Syn-associated nucleation processes and structural species related to neurotoxicity. Our findings revealed that BRICHOS predominately suppresses the formation of new nucleation units on the fibrils surface (secondary nucleation), in addition to fibril-end elongation. This mechanism implies a drastic decrease of the oligomer generation rate. Besides targeting secondary nucleation sites on the fibril surface, BRICHOS directly binds to oligomeric Syn species. Further, using ex vivo experiments, BRICHOS effectively diminishes Syn fibril-related toxicity to hippocampal electrophysiology. Our studies show that molecular chaperones can be utilized as tools to target molecular processes and structural species related to Syn neurotoxicity and have the potential as protein-based treatments against neurodegenerative disorders.

biophysics↗