ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43

Mechanism for transforming cytosolic SOD1 into integral membrane proteins of organelles by ALS-causing mutations

(2015) Liangzhong Lim et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

NMR and MD Studies Reveal That the Isolated Dengue NS3 Protease Is an Intrinsically Disordered Chymotrypsin Fold Which Absolutely Requests NS2B for Correct Folding and Functional Dynamics

(2015) Garvita Gupta et al.   PLoS One

Dynamic Principle for Designing Antagonistic/Agonistic Molecules for EphA4 Receptor, the Only Known ALS Modifier

(2014) Haina Qin et al.   ACS Chemical Biology

TDP-43 is a key player in the clinical features associated with Alzheimer’s disease

(2014) Keith A. Josephs et al.   ACTA NEUROPATHOLOGICA

“Structural characterization of the minimal segment of TDP-43 competent for aggregation”

(2014) Miguel Mompeán et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Differences between amyloid-β aggregation in solution and on the membrane: insights into elucidation of the mechanistic details of Alzheimer's disease

(2014) Samuel A. Kotler et al.   CHEMICAL SOCIETY REVIEWS

Controlling entropy to tune the functions of intrinsically disordered regions

(2014) Tilman Flock et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs

(2014) Hiroaki Suzuki et al.   HUMAN MOLECULAR GENETICS

Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease

(2014) Kuan-Yu Liu et al.   HUMAN MOLECULAR GENETICS

An ALS-mutant TDP-43 neurotoxic peptide adopts an anti-parallel β-structure and induces TDP-43 redistribution

(2014) Li Zhu et al.   HUMAN MOLECULAR GENETICS

Assemblages: Functional units formed by cellular phase separation

(2014) Jeffrey A. Toretsky et al.   JOURNAL OF CELL BIOLOGY

In Search of Aggregation Pathways of IAPP and Other Amyloidogenic Proteins: Finding Answers through NMR Spectroscopy

(2014) Hiren R. Patel et al.   Journal of Physical Chemistry Letters

Aggregate formation prevents dTDP-43 neurotoxicity in the Drosophila melanogaster eye

(2014) Lucia Cragnaz et al.   NEUROBIOLOGY OF DISEASE

TDP-43 N terminus encodes a novel ubiquitin-like fold and its unfolded form in equilibrium that can be shifted by binding to ssDNA

(2014) Haina Qin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Protein amyloids develop an intrinsic fluorescence signature during aggregation

(2013) Fiona T. S. Chan et al.   ANALYST

Membrane Interactions of the Amphipathic Amino Terminus of Huntingtin

(2013) Matthias Michalek et al.   BIOCHEMISTRY

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

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

Delineating the membrane-disrupting and seeding properties of the TDP-43 amyloidogenic core

(2013) Gerard Chun-Hao Liu et al.   CHEMICAL COMMUNICATIONS

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation

(2013) Yong-Jie Zhang et al.   HUMAN MOLECULAR GENETICS

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

Stress granules as crucibles of ALS pathogenesis

(2013) Yun R. Li et al.   JOURNAL OF CELL BIOLOGY

Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43

(2013) Peter J Lukavsky et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

(2013) Shuo-Chien Ling et al.   NEURON

Membrane disordering is not sufficient for membrane permeabilization by islet amyloid polypeptide: studies of IAPP(20–29) fragments

(2013) Jeffrey R. Brender et al.   PHYSICAL CHEMISTRY CHEMICAL PHYSICS

RNA Seeds Higher-Order Assembly of FUS Protein

(2013) Jacob C. Schwartz et al.   Cell Reports

Two-Step Mechanism of Membrane Disruption by Aβ through Membrane Fragmentation and Pore Formation

(2012) Michele F.M. Sciacca et al.   BIOPHYSICAL JOURNAL

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

The Magic of Bicelles Lights Up Membrane Protein Structure

(2012) Ulrich H. N. Dürr et al.   CHEMICAL REVIEWS

Accelerated Disease Onset with Stabilized Familial Amyotrophic Lateral Sclerosis (ALS)-linked Mutant TDP-43 Proteins

(2012) Shoji Watanabe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region

(2012) Mauricio Budini et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Biomolecular dynamics: order–disorder transitions and energy landscapes

(2012) Paul C Whitford et al.   REPORTS ON PROGRESS IN PHYSICS

The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies

(2012) I.-Fan Wang et al.   Nature Communications

Membrane Disruption and Early Events in the Aggregation of the Diabetes Related Peptide IAPP from a Molecular Perspective

(2011) Jeffrey R. Brender et al.   ACCOUNTS OF CHEMICAL RESEARCH

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou et al.   CELL

Molecular Dissection of TDP-43 Proteinopathies

(2011) Masato Hasegawa et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

(2011) Weirui Guo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS

(2011) Zhihui Sun et al.   PLOS BIOLOGY

Elimination of the Native Structure and Solubility of the hVAPB MSP Domain by the Pro56Ser Mutation That Causes Amyotrophic Lateral Sclerosis

(2010) Jiahai Shi et al.   BIOCHEMISTRY

Interaction with Polyglutamine Aggregates Reveals a Q/N-rich Domain in TDP-43

(2010) Rodrigo A. Fuentealba et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Design of Short Linear Peptides That Show Hydrogen Bonding Constraints in Water

(2010) Benben Song et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS

(2010) S.-C. Ling et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Systematic Survey Identifies Prions and Illuminates Sequence Features of Prionogenic Proteins

(2009) Simon Alberti et al.   CELL

Insight into “insoluble proteins” with pure water

(2009) Jianxing Song   FEBS LETTERS

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

Solution-state characteristics of the ultraviolet A-induced visible fluorescence from proteins

(2008) Purnananda Guptasarma   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

(2008) Matthew J. Winton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy

(2008) Julian R. Thorpe et al.   JOURNAL OF NEURAL TRANSMISSION

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

(2008) C. Vande Velde et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA