Zebrafish Study Links Somatic TARDBP Variants to FTLD-TDP-C
A study in Acta Neuropathologica Communications demonstrates that somatic TARDBP variants linked to FTLD-TDP-C disrupt morphology and increase microglia in ...
Tag: TDP-43
TDP-43 Hijacks a Tankyrase Switch That Powers Proteasome Assembly and Axonal Transport
A new study shows that pathological TDP-43 sequesters tankyrase into insoluble complexes, disrupting PI31-mediated proteasome assembly and axonal transport in ...
Sleep loss after brain injury boosts early Alzheimer-like protein changes in mice
A new mouse study shows that traumatic brain injury alone triggers early Alzheimer-like protein variant buildup in wild-type animals, and ...
TBK1 clears aggregation-prone TDP-43 via an IFNβ-immunoproteasome pathway in ALS models
Researchers report that the ALS-linked kinase TBK1 clears aggregation-prone monomeric TDP-43 in neurons by inducing interferon-β and the immunoproteasome, an ...
TDP-43 Overexpression in Human Cells Reproduces Key ALS Pathology and Gene Changes
Scientists built an inducible human cell line that overproduces TDP-43, triggering toxicity, mislocalization, and gene expression changes that overlap with ...
Fly Model Links C9orf72 Repeats to Age-Dependent TDP-43 Pathology and Dementia-Like Phenotypes
A Drosophila study shows that expressing expanded C9orf72 G4C2 repeats in mushroom body neurons drives age-dependent TDP-43 mislocalization, synaptic loss ...
Blocked protein cleanup reroutes toxic TDP-43 cargo through extracellular vesicles
In motor neuron–like cells, inhibiting the proteasome or autophagy reshapes extracellular vesicle cargo and spreads proteotoxic stress to recipient cells.
Astrocyte Lipid Metabolism Emerges as a Central Player in ALS Progression
A new review argues that disrupted lipid handling in astrocytes may amplify motor neuron injury and disease progression in ALS.
Progranulin Loss Impairs Cathepsin D Activity and Raises Insoluble TDP-43 in Mouse Brains
A mouse study finds that progranulin deficiency impairs cathepsin D enzymatic activity despite elevated protein levels, disrupts lysosomal organization, and ...
Experimental ALS study identifies interferon-beta immunoproteasome pathway for TDP-43 clearance
An experimental study in ALS models found that an interferon-beta signal can increase motor-neuron immunoproteasome activity and clear aggregation-prone TDP-43 ...
AI-Designed Enzymes Slice Disease Proteins With Unprecedented Precision
Researchers have used AI-driven protein design to create entirely new zinc-dependent proteases that cleave disease-linked proteins such as TDP-43 and ...
Cellular Bubbles and Molecular Trash Tags: A New Route for Antibody Drugs Into the Brain
University of Ottawa researchers report that extracellular vesicles can ferry engineered antibodies across the blood-brain barrier, and that tagging disease ...
Rare Prion Disease VPSPr Hides Among Other Proteinopathies, Two New Cases Reveal
Two new Austrian autopsy cases of variably protease-sensitive prionopathy reveal how codon 129 genetics and overlapping proteinopathies complicate diagnosis of ...
ALS’s ‘Lateral Sclerosis’ Revealed as a Dying-Back Degeneration of Upper Motor Neuron Axons
A clinical-neuropathological study shows that the lateral sclerosis of ALS reflects a length-dependent dying-back degeneration of upper motor neuron axons, ...
TDP-43 Rides Multiple Molecular Motors Along Human Axons, Study Reveals
New research in human neurons shows that the ALS-linked RNA-binding protein TDP-43 is transported along axons by a flexible fleet ...
Heart Disease Genetics: Little-Known Chaperone Hero11 Emerges as Coronary Risk Gene
Genetic variants in the heat-resistant obscure chaperone protein Hero11, encoded by C19orf53, are associated with coronary artery disease risk and ...
How one assay turned prion detection into a revolution across neurodegeneration
A new review in Acta Neuropathologica chronicles how Byron Caughey's development of the RT-QuIC assay revolutionized prion detection and seeded ...
Beyond ALS: Misfolded SOD1 Emerges as a Shared Player in Brain Disease
A sweeping review of human neuropathological evidence shows that misfolded SOD1, long tied to familial ALS, coexists with TDP-43, tau, ...
TDP-43 Loss Triggers Broad Polyadenylation Shifts in FTD/ALS
In a groundbreaking study published in Nature Neuroscience, researchers have unveiled that the nuclear depletion of the protein TDP-43 in ...
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