4.8 Article

Sequence Determines the Switch in the Fibril Forming Regions in the Low-Complexity FUS Protein and Its Variants

相关参考文献

注意:仅列出部分参考文献,下载原文获取全部文献信息。
Article Biochemistry & Molecular Biology

Insights into amyotrophic lateral sclerosis linked Pro525Arg mutation in the fused in sarcoma protein throughin silicoanalysis and molecular dynamics simulation

Sompriya Chatterjee et al.

Summary: This study investigated the impact of a Pro525Arg mutation in the FUS protein on stability and function, revealing that the mutation results in a less stable and more compact protein structure. Furthermore, the mutation alters the nuclear function of FUS, potentially leading to its accumulation in the cytoplasm.

JOURNAL OF BIOMOLECULAR STRUCTURE & DYNAMICS (2021)

Article Chemistry, Physical

Simulation of FUS Protein Condensates with an Adapted Coarse-Grained Model

Zakarya Benayad et al.

Summary: In this study, researchers developed a strategy to fine-tune the potential energy function for molecular dynamics simulations of biopolymer phase separation. By rebalancing protein-protein interactions against solvation and entropic contributions, they were able to simulate the liquid droplet formation of FUS low-complexity domain (LCD) with a rebalanced MARTINI model. The simulations successfully captured the phase separation of FUS-LCD droplets and provided insights into their physicochemical properties and dynamics.

JOURNAL OF CHEMICAL THEORY AND COMPUTATION (2021)

Article Biophysics

Real-time observation of structure and dynamics during the liquid-to-solid transition of FUS LC

Raymond F. Berkeley et al.

Summary: Proteins found in pathological fibrils can exhibit liquid-liquid phase separation tendencies, with real-time tracking revealing structural transitions in RNA-binding protein FUS. Disease-relevant mutations have significant effects on the process.

BIOPHYSICAL JOURNAL (2021)

Article Biochemistry & Molecular Biology

NMR and EPR reveal a compaction of the RNA-binding protein FUS upon droplet formation

Leonidas Emmanouilidis et al.

Summary: The study demonstrates the stabilization of liquid droplets of FUS protein using agarose hydrogel for spectroscopic characterization. The agarose hydrogel acts as a cytoskeleton mimic and cryoprotectant, enabling precise quantification of protein signals in both dispersed and condensed phases, even at cryogenic temperatures. Surprisingly, compact FUS protein was observed in the condensed phase using double electron-electron resonance measurements.

NATURE CHEMICAL BIOLOGY (2021)

Article Multidisciplinary Sciences

Reentrant liquid condensate phase of proteins is stabilized by hydrophobic and non-ionic interactions

Georg Krainer et al.

Summary: The study demonstrates that cellular proteins that form condensates at low salt concentrations can reenter a phase-separated regime at high salt concentrations, driven by hydrophobic and non-ionic interactions. This sheds light on the cooperation of hydrophobic and non-ionic interactions as general driving forces in the condensation process, with implications for biomolecular condensates.

NATURE COMMUNICATIONS (2021)

Article Chemistry, Multidisciplinary

Identification of the Rigid Core for Aged Liquid Droplets of an RNA-Binding Protein Low Complexity Domain

Blake D. Fonda et al.

Summary: The biomolecular condensation of proteins with low complexity sequences has functional roles in RNA metabolism but can be pathogenic in neurodegenerative diseases. The transformation of dynamic liquid droplets aids in achieving complex cellular functions, but the solidification of these droplets into beta-strand-rich hydrogel structures is mainly considered pathological. Low complexity sequences often contain multiple fibril-prone regions that can have both functional and pathological interactions, with one region stabilizing beta-strand-rich structure inhibiting another region from adopting a rigid fibril structure.

JOURNAL OF THE AMERICAN CHEMICAL SOCIETY (2021)

Article Multidisciplinary Sciences

Synaptic FUS accumulation triggers early misregulation of synaptic RNAs in a mouse model of ALS

Sonu Sahadevan et al.

Summary: Mutations in the RNA-binding protein FUS disrupt its nuclear localization and contribute to amyotrophic lateral sclerosis. Accumulation of synaptic FUS in early disease leads to synaptic impairment, potentially representing an initial trigger of neurodegeneration. The study identifies proteins associated with synapse organization and plasticity that are differentially regulated in an ALS mouse model.

NATURE COMMUNICATIONS (2021)

Article Chemistry, Physical

Model for disordered proteins with strongly sequence-dependent liquid phase behavior

Antonia Statt et al.

JOURNAL OF CHEMICAL PHYSICS (2020)

Article Biochemistry & Molecular Biology

Amyloid-Forming Segment Induces Aggregation of FUS-LC Domain from Phase Separation Modulated by Site-Specific Phosphorylation

Xiufang Ding et al.

JOURNAL OF MOLECULAR BIOLOGY (2020)

Article Chemistry, Physical

Biomolecular Phase Separation: From Molecular Driving Forces to Macroscopic Properties

Gregory L. Dignon et al.

Annual Review of Physical Chemistry (2020)

Article Chemistry, Physical

Single-Protein Collapse Determines Phase Equilibria of a Biological Condensate

Han-Yi Chou et al.

JOURNAL OF PHYSICAL CHEMISTRY LETTERS (2020)

Article Multidisciplinary Sciences

Differences in the free energies between the excited states of Aβ40 and Aβ42 monomers encode their aggregation propensities

Debayan Chakraborty et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2020)

Article Chemistry, Physical

Sequence Effects on Size, Shape, and Structural Heterogeneity in Intrinsically Disordered Proteins

Upayan Baul et al.

JOURNAL OF PHYSICAL CHEMISTRY B (2019)

Article Biochemistry & Molecular Biology

Molecular interactions underlying liquid-liquid phase separation of the FUS low-complexity domain

Anastasia C. Murthy et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2019)

Review Biochemistry & Molecular Biology

Studies on Molecular Dynamics of Intrinsically Disordered Proteins and Their Fuzzy Complexes: A Mini-Review

Kota Kasahara et al.

COMPUTATIONAL AND STRUCTURAL BIOTECHNOLOGY JOURNAL (2019)

Article Biochemistry & Molecular Biology

Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation

Mario Hofweber et al.

Article Biochemistry & Molecular Biology

Self-assembly of FUS through its low-complexity domain contributes to neurodegeneration

Taisei Matsumoto et al.

HUMAN MOLECULAR GENETICS (2018)

Article Chemistry, Physical

A Lattice Model of Charge-Pattern-Dependent Polyampholyte Phase Separation

Suman Das et al.

JOURNAL OF PHYSICAL CHEMISTRY B (2018)

Article Biochemistry & Molecular Biology

Atomic structures of FUS LC domain segments reveal bases for reversible amyloid fibril formation

Feng Luo et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2018)

Article Biochemistry & Molecular Biology

Atomic structures of FUS LC domain segments reveal bases for reversible amyloid fibril formation

Feng Luo et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2018)

Article Biochemical Research Methods

Sequence determinants of protein phase behavior from a coarse-grained model

Gregory L. Dignon et al.

PLOS COMPUTATIONAL BIOLOGY (2018)

Article Biochemistry & Molecular Biology

Phosphorylation of the FUS low-complexity domain disrupts phase separation, aggregation, and toxicity

Zachary Monahan et al.

EMBO JOURNAL (2017)

Article Biochemistry & Molecular Biology

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

Avinash Patel et al.

Article Biochemistry & Molecular Biology

Residue-by-Residue View of In Vitro FUS Granules that Bind the C-Terminal Domain of RNA Polymerase II

Kathleen A. Burke et al.

MOLECULAR CELL (2015)

Article Biochemistry & Molecular Biology

Nucleic acid-binding specificity of human FUS protein

Xueyin Wang et al.

NUCLEIC ACIDS RESEARCH (2015)

Article Biochemistry & Molecular Biology

Propensity to Form Amyloid Fibrils Is Encoded as Excitations in the Free Energy Landscape of Monomeric Proteins

Pavel I. Zhuravlev et al.

JOURNAL OF MOLECULAR BIOLOGY (2014)

Article Multidisciplinary Sciences

Structure of an Intermediate State in Protein Folding and Aggregation

Philipp Neudecker et al.

SCIENCE (2012)

Review Chemistry, Physical

Toward a Molecular Theory of Early and Late Events in Monomer to Amyloid Fibril Formation

John E. Straub et al.

ANNUAL REVIEW OF PHYSICAL CHEMISTRY, VOL 62 (2011)

Article Physics, Multidisciplinary

Factors Governing Fibrillogenesis of Polypeptide Chains Revealed by Lattice Models

Mai Suan Li et al.

PHYSICAL REVIEW LETTERS (2010)

Article Multidisciplinary Sciences

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

T. J. Kwiatkowski et al.

SCIENCE (2009)

Article Chemistry, Multidisciplinary

Dynamics of Asp23-Lys28 salt-bridge formation in Aβ10-35 monomers

Bogdan Tarus et al.

JOURNAL OF THE AMERICAN CHEMICAL SOCIETY (2006)