Granulins modulate liquid–liquid phase separation and aggregation of the prion-like C-terminal domain of the neurodegeneration-associated protein TDP-43

Aggregation of the nucleic acid–binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation

(2019) Youjun Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis

(2019) Archana Prasad et al.   Frontiers in Molecular Neuroscience

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

Tau and TDP-43 proteinopathies: kindred pathologic cascades and genetic pleiotropy

(2019) Yevgen Chornenkyy et al.   LABORATORY INVESTIGATION

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

Cysteine-rich granulin-3 rapidly promotes amyloid-β fibrils in both redox states

(2019) Anukool A. Bhopatkar et al.   BIOCHEMICAL JOURNAL

Multi-Granulin Domain Peptides Bind to Pro-Cathepsin D and Stimulate Its Enzymatic Activity More Effectively Than Progranulin in Vitro

(2019) Victoria J. Butler et al.   BIOCHEMISTRY

Liquid-liquid phase separation of tau protein: The crucial role of electrostatic interactions

(2019) Solomiia Boyko et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Aqueous Liquid-Liquid Phase Separation of Natural and Synthetic Polyguanidiniums

(2019) Prather et al.   Polymers

The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD

(2019) Britt A. Berning et al.   Frontiers in Neuroscience

Partial Failure of Proteostasis Systems Counteracting TDP-43 Aggregates in Neurodegenerative Diseases

(2019) Roberta Cascella et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The Role of TDP-43 in Military-Relevant TBI and Chronic Neurodegeneration

(2019) Lanier Heyburn et al.   Frontiers in Neurology

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

(2018) Jie Wang et al.   CELL

mTORC1 Controls Phase Separation and the Biophysical Properties of the Cytoplasm by Tuning Crowding

(2018) M. Delarue et al.   CELL

A single N‐terminal phosphomimic disrupts TDP‐43 polymerization, phase separation, and RNA splicing

(2018) Ailin Wang et al.   EMBO JOURNAL

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

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

IUPred2A: context-dependent prediction of protein disorder as a function of redox state and protein binding

(2018) Bálint Mészáros et al.   NUCLEIC ACIDS RESEARCH

TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

(2018) Yousra Khalfallah et al.   Scientific Reports

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

(2018) Sonja Kroschwald et al.   Cell Reports

Protein Phase Separation Provides Long-Term Memory of Transient Spatial Stimuli

(2018) Elliot Dine et al.   Cell Systems

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

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

Quantitative analysis of multilayer organization of proteins and RNA in nuclear speckles at super resolution

(2017) Jingyi Fei et al.   JOURNAL OF CELL SCIENCE

Disease and Region Specificity of Granulin Immunopositivities in Alzheimer Disease and Frontotemporal Lobar Degeneration

(2017) Qinwen Mao et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Numerous interactions act redundantly to assemble a tunable size of P bodies inSaccharomyces cerevisiae

(2017) Bhalchandra S. Rao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prediction of protein disorder based on IUPred

(2017) Zsuzsanna Dosztányi   PROTEIN SCIENCE

Disulfide bonds and disorder in granulin-3: An unusual handshake between structural stability and plasticity

(2017) Gaurav Ghag et al.   PROTEIN SCIENCE

Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation

(2017) Tariq Afroz et al.   Nature Communications

Compositional Control of Phase-Separated Cellular Bodies

(2016) Salman F. Banani et al.   CELL

Phase separation in biology; functional organization of a higher order

(2016) Diana M. Mitrea et al.   Cell Communication and Signaling

Intrinsic disorder in proteins involved in amyotrophic lateral sclerosis

(2016) Nikolas Santamaria et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Sequence Determinants of Intracellular Phase Separation by Complex Coacervation of a Disordered Protein

(2016) Chi W. Pak et al.   MOLECULAR CELL

Fully reduced granulin-B is intrinsically disordered and displays concentration-dependent dynamics

(2016) Gaurav Ghag et al.   PROTEIN ENGINEERING DESIGN & SELECTION

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

A Concentration-Dependent Liquid Phase Separation Can Cause Toxicity upon Increased Protein Expression

(2016) Benedetta Bolognesi et al.   Cell Reports

Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies

(2016) Lin Guo et al.   Cold Spring Harbor Perspectives in Medicine

Interaction of RNA with a C-terminal fragment of the amyotrophic lateral sclerosis-associated TDP43 reduces cytotoxicity

(2016) Akira Kitamura et al.   Scientific Reports

Relating sequence encoded information to form and function of intrinsically disordered proteins

(2015) Rahul K Das et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

The Progranulin Cleavage Products, Granulins, Exacerbate TDP-43 Toxicity and Increase TDP-43 Levels

(2015) D. A. Salazar et al.   JOURNAL OF NEUROSCIENCE

Frontotemporal dementia

(2015) Jee Bang et al.   LANCET

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

The Role of TDP-43 in Alzheimer’s Disease

(2015) Xiao-Long Chang et al.   MOLECULAR NEUROBIOLOGY

Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine

(2014) David J. Irwin et al.   ACTA NEUROPATHOLOGICA

Liquid-Liquid Phase Separation in Biology

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

Comprehensive large-scale assessment of intrinsic protein disorder

(2014) Ian Walsh et al.   BIOINFORMATICS

TDP-43 Inclusion Bodies Formed in Bacteria Are Structurally Amorphous, Non-Amyloid and Inherently Toxic to Neuroblastoma Cells

(2014) Claudia Capitini et al.   PLoS One

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

(2013) Ilmin Kwon et al.   CELL

Structural Transformation of the Amyloidogenic Core Region of TDP-43 Protein Initiates Its Aggregation and Cytoplasmic Inclusion

(2013) Lei-Lei Jiang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Accurate prediction of disorder in protein chains with a comprehensive and empirically designed consensus

(2013) Xiao Fan et al.   JOURNAL OF BIOMOLECULAR STRUCTURE & DYNAMICS

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Mechanisms of Granulin Deficiency: Lessons from Cellular and Animal Models

(2012) Gernot Kleinberger et al.   MOLECULAR NEUROBIOLOGY

A harmonized classification system for FTLD-TDP pathology

(2011) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1

(2011) Karli K. McDonald et al.   HUMAN MOLECULAR GENETICS

A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions

(2011) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

(2011) James R Tollervey et al.   NATURE NEUROSCIENCE

PONDR-FIT: A meta-predictor of intrinsically disordered amino acids

(2010) Bin Xue et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Molecular mechanism of Thioflavin-T binding to amyloid fibrils

(2010) Matthew Biancalana et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Progranulin expression in the developing and adult murine brain

(2010) Terri L. Petkau et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Serum biomarker for progranulin-associated frontotemporal lobar degeneration

(2009) Kristel Sleegers et al.   ANNALS OF NEUROLOGY

ANCHOR: web server for predicting protein binding regions in disordered proteins

(2009) Z. Dosztanyi et al.   BIOINFORMATICS

Frequency-specific modulation of population-level frequency tuning in human auditory cortex

(2009) Hidehiko Okamoto et al.   BMC NEUROSCIENCE

Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members

(2009) N. Finch et al.   BRAIN

Predicting intrinsic disorder in proteins: an overview

(2009) Bo He et al.   CELL RESEARCH

TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity

(2009) Brian S. Johnson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

(2009) Y.-J. Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prediction of Protein Binding Regions in Disordered Proteins

(2009) Bálint Mészáros et al.   PLoS Computational Biology

Enrichment of C-Terminal Fragments in TAR DNA-Binding Protein-43 Cytoplasmic Inclusions in Brain but not in Spinal Cord of Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

(2008) Lionel M. Igaz et al.   AMERICAN JOURNAL OF PATHOLOGY

Gene expression study on peripheral blood identifies progranulin mutations

(2008) Giovanni Coppola et al.   ANNALS OF NEUROLOGY

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2008) Masato Hasegawa et al.   ANNALS OF NEUROLOGY

MeDor: a metaserver for predicting protein disorder

(2008) Philippe Lieutaud et al.   BMC GENOMICS

Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury

(2008) Katie Moisse et al.   BRAIN RESEARCH

Structural determinants of the cellular localization and shuttling of TDP-43

(2008) Y. M. Ayala et al.   JOURNAL OF CELL SCIENCE

Low plasma progranulin levels predict progranulin mutations in frontotemporal lobar degeneration

(2008) R. Ghidoni et al.   NEUROLOGY

Structure dissection of human progranulin identifies well-folded granulin/epithelin modules with unique functional activities

(2008) Dmitri Tolkatchev et al.   PROTEIN SCIENCE