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Elevated nuclear TDP-43 induces constitutive exon skip**
BackgroundCytoplasmic inclusions and loss of nuclear TDP-43 are key pathological features found in several neurodegenerative disorders, suggesting...
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Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies
Frontotemporal lobar degeneration (FTLD) is a group of disorders characterized by degeneration of the frontal and temporal lobes, leading to...
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TDP-43-regulated cryptic RNAs accumulate in Alzheimer’s disease brains
BackgroundInclusions of TAR DNA-binding protein 43 kDa (TDP-43) has been designated limbic-predominant, age-related TDP-43 encephalopathy (LATE),...
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Seeding the aggregation of TDP-43 requires post-fibrillization proteolytic cleavage
Despite the strong evidence linking the transactive response DNA-binding protein 43 (TDP-43) aggregation to the pathogenesis of frontotemporal lobar...
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In vivo diagnosis of TDP-43 proteinopathies: in search of biomarkers of clinical use
TDP-43 proteinopathies are a heterogeneous group of neurodegenerative disorders that share the presence of aberrant, misfolded and mislocalized...
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Plasma extracellular vesicle tau and TDP-43 as diagnostic biomarkers in FTD and ALS
Minimally invasive biomarkers are urgently needed to detect molecular pathology in frontotemporal dementia (FTD) and amyotrophic lateral sclerosis...
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TDP-43 pathology is associated with increased tau burdens and seeding
BackgroundMost Alzheimer’s Disease (AD) cases also exhibit limbic predominant age-related TDP-43 encephalopathy neuropathological changes (LATE-NC),...
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HDGFL2 cryptic proteins report presence of TDP-43 pathology in neurodegenerative diseases
This letter demonstrates the potential of novel cryptic proteins resulting from TAR DNA-binding protein 43 (TDP-43) dysfunction as markers of TDP-43...
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Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and...
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Evidence of cerebellar TDP-43 loss of function in FTLD-TDP
Frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) is a neurodegenerative disease primarily affecting the frontal and/or temporal...
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A fluid biomarker reveals loss of TDP-43 splicing repression in presymptomatic ALS–FTD
Although loss of TAR DNA-binding protein 43 kDa (TDP-43) splicing repression is well documented in postmortem tissues of amyotrophic lateral...
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Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia
BackgroundProgranulin (PGRN) haploinsufficiency due to progranulin gene ( GRN ) variants can cause frontotemporal dementia (FTD) with aberrant TAR...
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Targeting RACK1 to alleviate TDP-43 and FUS proteinopathy-mediated suppression of protein translation and neurodegeneration
TAR DNA-binding protein 43 (TDP-43) and Fused in Sarcoma/Translocated in Sarcoma (FUS) are ribonucleoproteins associated with pathogenesis of...
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Specific vulnerability of iPSC-derived motor neurons with TDP-43 gene mutation to oxidative stress
Amyotrophic lateral sclerosis (ALS) is a disease that affects motor neurons and has a poor prognosis. We focused on TAR DNA-binding protein 43 kDa...
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Mis-localization of endogenous TDP-43 leads to ALS-like early-stage metabolic dysfunction and progressive motor deficits
BackgroundThe key pathological signature of ALS/ FTLD is the mis-localization of endogenous TDP-43 from the nucleus to the cytoplasm. However, TDP-43...
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TREM2 interacts with TDP-43 and mediates microglial neuroprotection against TDP-43-related neurodegeneration
Triggering receptor expressed on myeloid cell 2 (TREM2) is linked to risk of neurodegenerative disease. However, the function of TREM2 in...
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Degradation of neurodegenerative disease-associated TDP-43 aggregates and oligomers via a proteolysis-targeting chimera
BackgroundAmyotrophic lateral sclerosis (ALS) associated with TAR DNA-binding protein 43 (TDP-43) aggregation has been considered as a lethal and...
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Protein Disulfide Isomerase Endoplasmic Reticulum Protein 57 (ERp57) is Protective Against ALS-Associated Mutant TDP-43 in Neuronal Cells
Amyotrophic Lateral Sclerosis (ALS) is a severe neurodegenerative disease affecting motor neurons. Pathological forms of Tar-DNA binding protein-43...
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TDP-43 dysregulation and neuromuscular junction disruption in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a disease characterized by upper and lower motor neuron (MN) loss with a signature feature of cytoplasmic...
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Dysregulation of stress granule dynamics by DCTN1 deficiency exacerbates TDP-43 pathology in Drosophila models of ALS/FTD
The abnormal aggregation of TDP-43 into cytoplasmic inclusions in affected neurons is a major pathological hallmark of amyotrophic lateral sclerosis...
