Amyloid fibril formation of α-synuclein (αS) is associated with multiple neurodegenerative diseases, including Parkinson's disease (PD). Growing evidence suggests that progression of PD is linked to cell-to-cell propagation of αS fibrils, which leads to seeding of endogenous intrinsically disordered monomer via templated elongation and secondary nucleation. A molecular understanding of the seeding mechanism and driving interactions is crucial to inhibit progression of amyloid formation. Here, using relaxation-based solution NMR experiments designed to probe large complexes, we probe weak interactions of intrinsically disordered acetylated-αS (Ac-αS) monomers with seeding-competent Ac-αS fibrils and seeding-incompetent off-pathway oligomers to identify Ac-αS monomer residues at the binding interface. Under conditions that favor fibril elongation, we determine that the first 11 N-terminal residues on the monomer form a common binding site for both fibrils and off-pathway oligomers. Additionally, the presence of off-pathway oligomers within a fibril seeding environment suppresses seeded amyloid formation, as observed through thioflavin-T fluorescence experiments. This highlights that off-pathway αS oligomers can act as an auto-inhibitor against αS fibril elongation. Based on these data taken together with previous results, we propose a model in which Ac-αS monomer recruitment to the fibril is driven by interactions between the intrinsically disordered monomer N terminus and the intrinsically disordered flanking regions (IDR) on the fibril surface. We suggest that this monomer recruitment may play a role in the elongation of amyloid fibrils and highlight the potential of the IDRs of the fibril as important therapeutic targets against seeded amyloid formation.
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http://dx.doi.org/10.1073/pnas.2017452118 | DOI Listing |
Mol Cell
January 2025
Institute of Biochemistry and Molecular Biology, National Yang Ming Chiao Tung University, No. 155, Section 2, Li-nong Street, Taipei, Taiwan; Department of Life Sciences and Institute of Genome Sciences, National Yang Ming Chiao Tung University, No. 155, Section 2, Li-nong Street, Taipei, Taiwan; Institute of Biomedical Informatics, National Yang Ming Chiao Tung University, No. 155, Section 2, Li-nong Street, Taipei, Taiwan. Electronic address:
Intrinsically disordered regions (IDRs) of proteins can regulate function through phase separation. In a recent article in Nature, Garcia-Cabau et al. reveal that including or excluding a microexon within the IDR of CPEB4 alters its condensation properties, suggesting a potential mechanism underlying autism spectrum disorder.
View Article and Find Full Text PDFProc Natl Acad Sci U S A
January 2025
Department of General Surgery, The First Affiliated Hospital of University of Science and Technology of China, Key Laboratory of Immune Response and Immunotherapy, Center for Advanced Interdisciplinary Science Interdisciplinary Science & Biomedicine of Institute of Health and Medicine, Division of Life Sciences & Medicine, University of Science and Technology of China, Hefei 230027, Anhui, China.
The DNA-sensing protein cGAS plays a pivotal role in the innate immune response and pathogenesis of various diseases. DNA triggers liquid-liquid phase separation (LLPS) and enhances the enzymatic activity of cGAS. However, the regulatory mechanisms of the disordered N terminus remain unclear.
View Article and Find Full Text PDFCurr Opin Struct Biol
January 2025
Department of Medical Biochemistry and Microbiology, Uppsala University, BMC, Box 582, SE-75123 Uppsala, Sweden. Electronic address:
Protein-protein associations are often mediated by an intrinsically disordered protein region interacting with a folded domain in a coupled binding and folding reaction. Classic physical organic chemistry approaches together with structural biology have shed light on mechanistic aspects of such reactions. Further insight into general principles may be obtained by interpreting the results through an evolutionary lens.
View Article and Find Full Text PDFJ Chem Inf Model
January 2025
Max-Planck-Institut für Immunbiologie und Epigenetik (MPI-IE), Stübeweg 51, 79108 Freiburg im Breisgau, Germany.
Intrinsically disordered regions are found in most eukaryotic proteins and are enriched with positively and negatively charged residues. While it is often convenient to assume that these residues follow their model-compound p values, recent work has shown that local charge effects (charge regulation) can upshift or downshift side chain p values with major consequences for molecular function. Despite this, charge regulation is rarely considered when investigating disordered regions.
View Article and Find Full Text PDFMicrob Cell Fact
January 2025
Lab of Environmental and Life Sciences, University of Nova Gorica, Vipavska cesta 13, Nova Gorica, 5000, Slovenia.
Background: E. coli still remains the most commonly used organism to produce recombinant proteins in research labs. This condition is mirrored by the attention that researchers dedicate to understanding the biology behind protein expression, which is then exploited to improve the effectiveness of the technology.
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