Gel‐like inclusions of C‐terminal fragments of TDP‐43 sequester stalled proteasomes in neurons

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

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Cryo-EM structures of four polymorphic TDP-43 amyloid cores

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The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation

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

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TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

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Atomic structures of TDP-43 LCD segments and insights into reversible or pathogenic aggregation

(2018) Elizabeth L. Guenther et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Atomic structures of TDP-43 LCD segments and insights into reversible or pathogenic aggregation

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The molecular language of membraneless organelles

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TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle

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Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways

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ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure

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

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

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An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic mice

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C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration

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Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: Characterization of inhibition by nucleic acids and 4-aminoquinolines

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Motor Neuron-specific Disruption of Proteasomes, but Not Autophagy, Replicates Amyotrophic Lateral Sclerosis

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Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

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Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

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

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VCPMutations Causing Frontotemporal Lobar Degeneration Disrupt Localization of TDP-43 and Induce Cell Death

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Global Analysis of TDP-43 Interacting Proteins Reveals Strong Association with RNA Splicing and Translation Machinery

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The Ubiquitin-Proteasome Reporter GFPu Does Not Accumulate in Neurons of the R6/2 Transgenic Mouse Model of Huntington's Disease

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Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

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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

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

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MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification

(2008) Jürgen Cox et al.   NATURE BIOTECHNOLOGY