Article

Astrocytic expression of ALS-causative mutant FUS leads to TNFα-dependent neurodegeneration <i>in vivo</i>

2021-11-24

Abstract excerpt

<h4>ABSTRACT</h4> Genetic mutations that cause Amyotrophic Lateral Sclerosis (ALS), a progressively lethal motor neuron disease, are commonly found in ubiquitously expressed genes. In addition to direct defects within motor neurons, growing evidence suggests that dysfunction of non-neuronal cells is also an important driver of disease. Previously, we demonstrated that mutations in DNA/RNA binding protein Fused in...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 78d39f38-501e-5e41-9c6d-696dd7383fc1
DOI: 10.1101/2021.11.23.469650

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Astrocytic expression of ALS-causative mutant FUS leads to TNFα-dependent neurodegeneration <i>in vivo</i>DOI 10.1101/2021.11.23.469650Read

Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha.Semantic proximity Read

Increased FUS levels in astrocytes leads to astrocyte and microglia activation and neuronal deathSemantic proximity Read

Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition.Semantic proximity Read

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashionSemantic proximity Read

TNFα hinders FGF4 efficacy to mitigate ALS astrocyte dysfunction and cGAS-STING pathway-induced innate immune reactivitySemantic proximity Read

Activity-dependent FUS dysregulation disrupts synaptic homeostasisSemantic proximity Read

FABP7 upregulation induces a neurotoxic phenotype in astrocytes.Semantic proximity Read

Astrocytic FABP5 drives non-cell-autonomous oligodendrocyte injury in multiple system atrophy by promoting TNF signaling and ferroptotic stressSemantic proximity Read

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons.Semantic proximity Read

Knockout of reactive astrocyte activating factors slows disease progression in an ALS mouse modelSemantic proximity Read

FUS aggregation following ischemic stroke favors brain astrocyte activation through inducing excessive autophagy.Semantic proximity Read

Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.Semantic proximity Read