Results 111 to 120 of about 12,015 (253)

TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss [PDF]

open access: yes, 2017
Microglia coordinate various functions in the central nervous system ranging from removing synaptic connections, to maintaining brain homeostasis by monitoring neuronal function, and clearing protein aggregates across the lifespan.
Ali Jawaid   +82 more
core   +4 more sources

Altered Inflammatory Signature in a C9ORF72 ‐ALS iPSC‐Derived Motor Neuron and Microglia Coculture Model

open access: yesGlia, Volume 74, Issue 1, January 2026.
Established a C9ORF72 ALS microglia and motor neuron coculture model. Identified an altered inflammatory signature in C9ORF72 ALS microglia. Single‐cell RNA sequencing detected the removal of an LPS responsive microglia subpopulation. ABSTRACT Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disorder involving multiple cell types in ...
Yujing Gao   +9 more
wiley   +1 more source

Increased risk for other cancers in individuals with Ewing sarcoma and their relatives. [PDF]

open access: yes, 2019
BackgroundThere are few reports of the association of other cancers with Ewing sarcoma in patients and their relatives. We use a resource combining statewide genealogy and cancer reporting to provide unbiased risks.MethodsUsing a combined genealogy of 2 ...
Abbott, Diana   +9 more
core   +1 more source

Oligodendroglial Densities and Myelin Structure Are Altered in TDP‐43 Related Amyotrophic Lateral Sclerosis

open access: yesGlia, Volume 74, Issue 1, January 2026.
TDP‐43Q331K mice exhibit altered proliferative dynamics in oligodendrocytes and their precursors. Oligodendroglial cell death is evident in TDP‐43Q331K mice. TDP‐43Q331K mice display altered myelin reflectance and pathology in the spinal cord. ABSTRACT Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by the ...
Katherine N. Lewis   +8 more
wiley   +1 more source

Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms

open access: yesDisease Models & Mechanisms, 2018
In the past, amyotrophic lateral sclerosis (ALS) has been considered a ‘neurocentric’ disease; however, new evidence suggests that it should instead be looked at from a ‘multisystemic’ or ‘non-neurocentric’ point of view.
Orietta Pansarasa   +10 more
doaj   +1 more source

ALS-linked TDP-43M337V knock-in mice exhibit splicing deregulation without neurodegeneration

open access: yesMolecular Brain, 2020
Abnormal accumulation of TAR DNA-binding protein 43 (TDP-43), a DNA/RNA binding protein, is a pathological signature of amyotrophic lateral sclerosis (ALS).
Seiji Watanabe   +7 more
doaj   +1 more source

TDP‐43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models

open access: yesThe FEBS Journal, Volume 293, Issue 2, Page 348-384, January 2026.
The manuscript explores structural and functional features of TDP‐43 and its worm homologue, TDP‐1, highlighting conserved and divergent structural and functional features. Using genetically engineered C. elegans models, key pathological features of TDP‐43 proteinopathies—including aggregation, neurodegeneration, and motor deficits—are recapitulated ...
Ghulam Jeelani Pir   +7 more
wiley   +1 more source

A unique mouse model for investigating the properties of amyotrophic lateral sclerosis-associated protein TDP-43, by in utero electroporation [PDF]

open access: yes, 2013
TDP-43 is a discriminative protein that is found as intracellular aggregations in the neurons of the cerebral cortex and spinal cord of patients with amyotrophic lateral sclerosis (ALS); however, the mechanisms of neuron loss and its relation to the ...
Akamatsu Megumi   +14 more
core   +1 more source

Cognitive and Behavioral Features of Patients With Amyotrophic Lateral Sclerosis Who Are Carriers of the TARDBP Pathogenic Variant

open access: yesNeurology
Background and Objectives TARDBP patients are considered particularly prone to cognitive involvement, but no systematic studies of cognitive impairment in TARDBP patients are available.
C. Moglia   +17 more
semanticscholar   +1 more source

Induced pluripotent stem cell models of frontotemporal dementia [PDF]

open access: yes, 2016
The increasing prevalence of dementia in the ageing population combined with the lack of treatments and the burden on national healthcare systems globally, make dementia a public health priority.
Hardy, J, Preza, E, Warner, T, Wray, S
core  

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