Biomolecular condensates at the nexus of cellular stress, protein aggregation disease and ageing

Physical Principles Underlying the Complex Biology of Intracellular Phase Transitions

(2020) Jeong-Mo Choi et al.   Annual Review of Biophysics

Modulation of RNA Condensation by the DEAD-Box Protein eIF4A

(2020) Devin Tauber et al.   CELL

The (un)structural biology of biomolecular liquid-liquid phase separation using NMR spectroscopy

(2020) Anastasia C. Murthy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phase separation organizes the site of autophagosome formation

(2020) Yuko Fujioka et al.   NATURE

Valence and patterning of aromatic residues determine the phase behavior of prion-like domains

(2020) Erik W. Martin et al.   SCIENCE

Phase separation provides a mechanism to reduce noise in cells

(2020) A. Klosin et al.   SCIENCE

Optogenetic modulation of TDP-43 oligomerization accelerates ALS-related pathologies in the spinal motor neurons

(2020) Kazuhide Asakawa et al.   Nature Communications

Condensation of Ded1p Promotes a Translational Switch from Housekeeping to Stress Protein Production

(2020) Christiane Iserman et al.   CELL

Competing Protein-RNA Interaction Networks Control Multiphase Intracellular Organization

(2020) David W. Sanders et al.   CELL

G3BP1 Is a Tunable Switch that Triggers Phase Separation to Assemble Stress Granules

(2020) Peiguo Yang et al.   CELL

RNA-Induced Conformational Switching and Clustering of G3BP Drive Stress Granule Assembly by Condensation

(2020) Jordina Guillén-Boixet et al.   CELL

Composition-dependent thermodynamics of intracellular phase separation

(2020) Joshua A. Riback et al.   NATURE

Author Correction: Dynamics of oligomer populations formed during the aggregation of Alzheimer’s Aβ42 peptide

(2020) Thomas C. T. Michaels et al.   Nature Chemistry

α-Synuclein aggregation nucleates through liquid–liquid phase separation

(2020) Soumik Ray et al.   Nature Chemistry

RNA contributions to the form and function of biomolecular condensates

(2020) Christine Roden et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Head-to-Tail Polymerization in the Assembly of Biomolecular Condensates

(2020) Mariann Bienz   CELL

Protein condensates as aging Maxwell fluids

(2020) Louise Jawerth et al.   SCIENCE

Protein Phase Separation as a Stress Survival Strategy

(2019) Titus M. Franzmann et al.   Cold Spring Harbor Perspectives in Biology

Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase

(2019) James Shorter et al.   Cold Spring Harbor Perspectives in Biology

Phase Separation, Transition, and Autophagic Degradation of Proteins in Development and Pathogenesis

(2019) Zheng Wang et al.   TRENDS IN CELL BIOLOGY

Stoichiometry controls activity of phase-separated clusters of actin signaling proteins

(2019) Lindsay B. Case et al.   SCIENCE

RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43

(2019) Jacob R. Mann et al.   NEURON

Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

(2019) Fatima Gasset-Rosa et al.   NEURON

The proteostasis network and its decline in ageing

(2019) Mark S. Hipp et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The role of liquid-liquid phase separation in aggregation of the TDP-43 low complexity domain

(2019) W. Michael Babinchak et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Considerations and Challenges in Studying Liquid-Liquid Phase Separation and Biomolecular Condensates

(2019) Simon Alberti et al.   CELL

FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy

(2019) Lara Marrone et al.   ACTA NEUROPATHOLOGICA

Regulation of Transmembrane Signaling by Phase Separation

(2019) Lindsay B. Case et al.   Annual Review of Biophysics

C9orf72 Poly(PR) Dipeptide Repeats Disturb Biomolecular Phase Separation and Disrupt Nucleolar Function

(2019) Michael R. White et al.   MOLECULAR CELL

Spatial control of irreversible protein aggregation

(2019) Christoph Weber et al.   eLife

Defective ribosomal products challenge nuclear function by impairing nuclear condensate dynamics and immobilizing ubiquitin

(2019) Laura Mediani et al.   EMBO JOURNAL

Phase separation of 53 BP 1 determines liquid‐like behavior of DNA repair compartments

(2019) Sinan Kilic et al.   EMBO JOURNAL

Clustered telomeres in phase-separated nuclear condensates engage mitotic DNA synthesis through BLM and RAD52

(2019) Jaewon Min et al.   GENES & DEVELOPMENT

Liquid–Liquid Phase Separation Is Driven by Large-Scale Conformational Unwinding and Fluctuations of Intrinsically Disordered Protein Molecules

(2019) Anupa Majumdar et al.   Journal of Physical Chemistry Letters

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

(2019) Anastasia C. Murthy et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

The nucleolus functions as a phase-separated protein quality control compartment

(2019) F. Frottin et al.   SCIENCE

Liquid–Liquid Phase Separation in Disease

(2019) Simon Alberti et al.   Annual Review of Genetics

DEAD-box ATPases are global regulators of phase-separated organelles

(2019) Maria Hondele et al.   NATURE

Organization of Chromatin by Intrinsic and Regulated Phase Separation

(2019) Bryan A. Gibson et al.   CELL

Evaluating phase separation in live cells: diagnosis, caveats, and functional consequences

(2019) David T. McSwiggen et al.   GENES & DEVELOPMENT

The Control Centers of Biomolecular Phase Separation: How Membrane Surfaces, PTMs, and Active Processes Regulate Condensation

(2019) Wilton T. Snead et al.   MOLECULAR CELL

Loss of Dynamic RNA Interaction and Aberrant Phase Separation Induced by Two Distinct Types of ALS/FTD-Linked FUS Mutations

(2019) Amirhossein Ghanbari Niaki et al.   MOLECULAR CELL

HP1 reshapes nucleosome core to promote phase separation of heterochromatin

(2019) S. Sanulli et al.   NATURE

Cellular quality control by the ubiquitin-proteasome system and autophagy

(2019) Christian Pohl et al.   SCIENCE

Molecular structure in biomolecular condensates

(2019) Ivan Peran et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

A Solid-State Conceptualization of Information Transfer from Gene to Message to Protein

(2018) Masato Kato et al.   Annual Review of Biochemistry

Functional Implications of Intracellular Phase Transitions

(2018) Alex S. Holehouse et al.   BIOCHEMISTRY

Nuclear Import Receptor Inhibits Phase Separation of FUS through Binding to Multiple Sites

(2018) Takuya Yoshizawa et al.   CELL

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

(2018) Mario Hofweber et al.   CELL

A Molecular Grammar Governing the Driving Forces for Phase Separation of Prion-like RNA Binding Proteins

(2018) Jie Wang et al.   CELL

Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains

(2018) Lin Guo et al.   CELL

FUS Phase Separation Is Modulated by a Molecular Chaperone and Methylation of Arginine Cation-π Interactions

(2018) Seema Qamar et al.   CELL

Polyubiquitin chain-induced p62 phase separation drives autophagic cargo segregation

(2018) Daxiao Sun et al.   CELL RESEARCH

Tau protein liquid–liquid phase separation can initiate tau aggregation

(2018) Susanne Wegmann et al.   EMBO JOURNAL

Deubiquitylases USP5 and USP13 are recruited to and regulate heat-induced stress granules through their deubiquitylating activities

(2018) Xuan Xie et al.   JOURNAL OF CELL SCIENCE

Quality Control of Membraneless Organelles

(2018) Simon Alberti et al.   JOURNAL OF MOLECULAR BIOLOGY

Ubiquitin Modulates Liquid-Liquid Phase Separation of UBQLN2 via Disruption of Multivalent Interactions

(2018) Thuy P. Dao et al.   MOLECULAR CELL

Mechanistic View of hnRNPA2 Low-Complexity Domain Structure, Interactions, and Phase Separation Altered by Mutation and Arginine Methylation

(2018) Veronica H. Ryan et al.   MOLECULAR CELL

ZFAND1 Recruits p97 and the 26S Proteasome to Promote the Clearance of Arsenite-Induced Stress Granules

(2018) Ankit Turakhiya et al.   MOLECULAR CELL

A Liquid to Solid Phase Transition Underlying Pathological Huntingtin Exon1 Aggregation

(2018) Thomas R. Peskett et al.   MOLECULAR CELL

Protein-Based Inheritance: Epigenetics beyond the Chromosome

(2018) Zachary H. Harvey et al.   MOLECULAR CELL

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

(2018) Feng Luo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

RNA buffers the phase separation behavior of prion-like RNA binding proteins

(2018) Shovamayee Maharana et al.   SCIENCE

Atomic structures of low-complexity protein segments reveal kinked β sheets that assemble networks

(2018) Michael P. Hughes et al.   SCIENCE

Phase separation of a yeast prion protein promotes cellular fitness

(2018) Titus M. Franzmann et al.   SCIENCE

Why Do Disordered and Structured Proteins Behave Differently in Phase Separation?

(2018) Huan-Xiang Zhou et al.   TRENDS IN BIOCHEMICAL SCIENCES

mRNA Translation Gone Awry: Translation Fidelity and Neurological Disease

(2018) Mridu Kapur et al.   TRENDS IN GENETICS

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

(2018) Marcel Naumann et al.   Nature Communications

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

(2018) Feng Luo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Different Material States of Pub1 Condensates Define Distinct Modes of Stress Adaptation and Recovery

(2018) Sonja Kroschwald et al.   Cell Reports

Dual roles for ATP in the regulation of phase separated protein aggregates in Xenopus oocyte nucleoli

(2018) Michael H Hayes et al.   eLife

mTOR Regulates Phase Separation of PGL Granules to Modulate Their Autophagic Degradation

(2018) Gangming Zhang et al.   CELL

Who's In and Who's Out—Compositional Control of Biomolecular Condensates

(2018) Jonathon A. Ditlev et al.   JOURNAL OF MOLECULAR BIOLOGY

p62-dependent phase separation of patient-derived KEAP1 mutations and NRF2

(2018) E. W. Cloer et al.   MOLECULAR AND CELLULAR BIOLOGY

Cancer Mutations of the Tumor Suppressor SPOP Disrupt the Formation of Active, Phase-Separated Compartments

(2018) Jill J. Bouchard et al.   MOLECULAR CELL

Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization

(2018) Leeanne McGurk et al.   MOLECULAR CELL

Kinase-controlled phase transition of membraneless organelles in mitosis

(2018) Arpan Kumar Rai et al.   NATURE

A new era for understanding amyloid structures and disease

(2018) Matthew G. Iadanza et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Dynamic micellar oligomers of amyloid beta peptides play a crucial role in their aggregation mechanisms

(2018) Bertrand Morel et al.   PHYSICAL CHEMISTRY CHEMICAL PHYSICS

Liquid Nuclear Condensates Mechanically Sense and Restructure the Genome

(2018) Yongdae Shin et al.   CELL

Transcriptional Signatures of Aging

(2017) R. Stegeman et al.   JOURNAL OF MOLECULAR BIOLOGY

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

Phase separation drives heterochromatin domain formation

(2017) Amy R. Strom et al.   NATURE

RNA phase transitions in repeat expansion disorders

(2017) Ankur Jain et al.   NATURE

Liquid droplet formation by HP1α suggests a role for phase separation in heterochromatin

(2017) Adam G. Larson et al.   NATURE

Reducing the RNA binding protein TIA1 protects against tau-mediated neurodegeneration in vivo

(2017) Daniel J. Apicco et al.   NATURE NEUROSCIENCE

Biomolecular condensates: organizers of cellular biochemistry

(2017) Salman F. Banani et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons

(2017) Pallavi P. Gopal et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS

(2017) Prajwal Ciryam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Independent active and thermodynamic processes govern the nucleolus assembly in vivo

(2017) Hanieh Falahati et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural and hydrodynamic properties of an intrinsically disordered region of a germ cell-specific protein on phase separation

(2017) Jacob P. Brady et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATP as a biological hydrotrope

(2017) Avinash Patel et al.   SCIENCE

Liquid phase condensation in cell physiology and disease

(2017) Yongdae Shin et al.   SCIENCE

Liquid–liquid phase separation of the microtubule-binding repeats of the Alzheimer-related protein Tau

(2017) Susmitha Ambadipudi et al.   Nature Communications

Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function

(2017) Federica F. Morelli et al.   Cell Reports

Granulostasis: Protein Quality Control of RNP Granules

(2017) Simon Alberti et al.   Frontiers in Molecular Neuroscience

Cellular Homeostasis and Aging

(2016) F. Ulrich Hartl   Annual Review of Biochemistry

Are aberrant phase transitions a driver of cellular aging?

(2016) Simon Alberti et al.   BIOESSAYS

Epigenetic Mechanisms of Longevity and Aging

(2016) Payel Sen et al.   CELL

The Structure and Dynamics of Higher-Order Assemblies: Amyloids, Signalosomes, and Granules

(2016) Hao Wu et al.   CELL

Polar Positioning of Phase-Separated Liquid Compartments in Cells Regulated by an mRNA Competition Mechanism

(2016) Shambaditya Saha et al.   CELL

ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure

(2016) Saumya Jain et al.   CELL

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

(2016) Kyung-Ha Lee et al.   CELL

Compositional Control of Phase-Separated Cellular Bodies

(2016) Salman F. Banani et al.   CELL

Adaptation to Stressors by Systemic Protein Amyloidogenesis

(2016) Timothy E. Audas et al.   DEVELOPMENTAL CELL

Mechanistic insights into mammalian stress granule dynamics

(2016) Marc D. Panas et al.   JOURNAL OF CELL BIOLOGY

A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone Complex Ensures Stress Granule Integrity and Dynamism

(2016) Massimo Ganassi et al.   MOLECULAR CELL

Ribosome-associated protein quality control

(2016) Onn Brandman et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Phase separation of signaling molecules promotes T cell receptor signal transduction

(2016) Xiaolei Su et al.   SCIENCE

ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain

(2016) Alexander E. Conicella et al.   STRUCTURE

Principles and Properties of Stress Granules

(2016) David S.W. Protter et al.   TRENDS IN CELL BIOLOGY

Cross-β Polymerization of Low Complexity Sequence Domains

(2016) Masato Kato et al.   Cold Spring Harbor Perspectives in Biology

The Role of Functional Prion-Like Proteins in the Persistence of Memory

(2016) Kausik Si et al.   Cold Spring Harbor Perspectives in Biology

Small heat shock proteins sequester misfolding proteins in near-native conformation for cellular protection and efficient refolding

(2016) Sophia Ungelenk et al.   Nature Communications

Ribosome-associated protein quality control

(2016) Onn Brandman et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A pH-driven transition of the cytoplasm from a fluid- to a solid-like state promotes entry into dormancy

(2016) Matthias Christoph Munder et al.   eLife

ATPase activity of the DEAD-box protein Dhh1 controls processing body formation

(2016) Christopher Frederick Mugler et al.   eLife

The Biology of Proteostasis in Aging and Disease

(2015) Johnathan Labbadia et al.   Annual Review of Biochemistry

Reversible, Specific, Active Aggregates of Endogenous Proteins Assemble upon Heat Stress

(2015) Edward W.J. Wallace et al.   CELL

Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization

(2015) Amandine Molliex et al.   CELL

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

(2015) Avinash Patel et al.   CELL

Compartment-specific aggregases direct distinct nuclear and cytoplasmic aggregate deposition

(2015) S. B. Miller et al.   EMBO JOURNAL

Structural, morphological, and functional diversity of amyloid oligomers

(2015) Leonid Breydo et al.   FEBS LETTERS

Elastin-like polypeptides as models of intrinsically disordered proteins

(2015) Stefan Roberts et al.   FEBS LETTERS

Spatially Organized Aggregation of Misfolded Proteins as Cellular Stress Defense Strategy

(2015) Stephanie B.M. Miller et al.   JOURNAL OF MOLECULAR BIOLOGY

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

(2015) Kathleen A. Burke et al.   MOLECULAR CELL

Phase Transition of a Disordered Nuage Protein Generates Environmentally Responsive Membraneless Organelles

(2015) Timothy J. Nott et al.   MOLECULAR CELL

Signalling scaffolds and local organization of cellular behaviour

(2015) Lorene K. Langeberg et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function

(2015) Tetsuro Murakami et al.   NEURON

A mechanism for the segregation of age in mammalian neural stem cells

(2015) D. L. Moore et al.   SCIENCE

Supersaturation is a major driving force for protein aggregation in neurodegenerative diseases

(2015) Prajwal Ciryam et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

Promiscuous interactions and protein disaggregases determine the material state of stress-inducible RNP granules

(2015) Sonja Kroschwald et al.   eLife

Liquid-Liquid Phase Separation in Biology

(2014) Anthony A. Hyman et al.   Annual Review of Cell and Developmental Biology

Free mRNA in excess upon polysome dissociation is a scaffold for protein multimerization to form stress granules

(2014) Ouissame Bounedjah et al.   NUCLEIC ACIDS RESEARCH

Protein disorder, prion propensities, and self-organizing macromolecular collectives

(2013) Liliana Malinovska et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

The Hallmarks of Aging

(2013) Carlos López-Otín et al.   CELL

Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function

(2013) J. Ross Buchan et al.   CELL

Phosphorylation-Regulated Binding of RNA Polymerase II to Fibrous Polymers of Low-Complexity Domains

(2013) Ilmin Kwon et al.   CELL

Altered Ribostasis: RNA-Protein Granules in Degenerative Disorders

(2013) Mani Ramaswami et al.   CELL

The Bacterial Cytoplasm Has Glass-like Properties and Is Fluidized by Metabolic Activity

(2013) Bradley R. Parry et al.   CELL

Coordination of Translational Control and Protein Homeostasis during Severe Heat Stress

(2013) Valeria Cherkasov et al.   CURRENT BIOLOGY

The asymmetric segregation of damaged proteins is stem cell–type dependent

(2013) Mary Rose Bufalino et al.   JOURNAL OF CELL BIOLOGY

Spatial sequestration of misfolded proteins by a dynamic chaperone pathway enhances cellular fitness during stress

(2013) Stéphanie Escusa-Toret et al.   NATURE CELL BIOLOGY

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease

(2012) Oliver D. King et al.   BRAIN RESEARCH

Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels

(2012) Masato Kato et al.   CELL

Cell-free Formation of RNA Granules: Bound RNAs Identify Features and Components of Cellular Assemblies

(2012) Tina W. Han et al.   CELL

Protein Misfolded Oligomers: Experimental Approaches, Mechanism of Formation, and Structure-Toxicity Relationships

(2012) Francesco Bemporad et al.   CHEMISTRY & BIOLOGY

Prions in Yeast

(2012) S. W. Liebman et al.   GENETICS

Molecular chaperones and stress-inducible protein-sorting factors coordinate the spatiotemporal distribution of protein aggregates

(2012) Liliana Malinovska et al.   MOLECULAR BIOLOGY OF THE CELL

Phase transitions in the assembly of multivalent signalling proteins

(2012) Pilong Li et al.   NATURE

Confinement to Organelle-Associated Inclusion Structures Mediates Asymmetric Inheritance of Aggregated Protein in Budding Yeast

(2012) Rachel Spokoini et al.   Cell Reports

Nucleolar aggresomes as counterparts of cytoplasmic aggresomes in proteotoxic stress

(2011) Leena Latonen   BIOESSAYS

Hsp42 is required for sequestration of protein aggregates into deposition sites inSaccharomyces cerevisiae

(2011) Sebastian Specht et al.   JOURNAL OF CELL BIOLOGY

Reversibility of β-Amyloid Self-Assembly: Effects of pH and Added Salts Assessed by Fluorescence Photobleaching Recovery

(2010) Nadia J. Edwin et al.   BIOMACROMOLECULES

Heat shock factors: integrators of cell stress, development and lifespan

(2010) Malin Åkerfelt et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

SEPA-1 Mediates the Specific Recognition and Degradation of P Granule Components by Autophagy in C. elegans

(2009) Yuxia Zhang et al.   CELL

Misfolded proteins partition between two distinct quality control compartments

(2008) Daniel Kaganovich et al.   NATURE

Intrinsically Disordered Proteins Display No Preference for Chaperone Binding In Vivo

(2008) Hedi Hegyi et al.   PLoS Computational Biology