ArticleHuman molecular genetics2012
Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.
Article in Human molecular genetics, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
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Who cites it
36 citing papers in PubMed, 60 citations in OpenAlex.
- Increase in hnRNPA1 Expression Suffices to Kill Motor Neurons in Transgenic Rats.International journal of molecular sciences · 2023Article
- Review
- The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS.International journal of molecular sciences · 2021Review
- Detergent-insoluble inclusion constitutes the first pathology in PFN1 transgenic rats.Journal of neurochemistry · 2021Article
- Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization.Nature communications · 2019Article
- Neuropathological changes and cognitive deficits in rats transgenic for human mutant tau recapitulate human tauopathy.Neurobiology of disease · 2019Article
- Motor Neuron Susceptibility in ALS/FTD.Frontiers in neuroscience · 2019Review
- Mutant UBQLN2Acta neuropathologica communications · 2018Article
- Amyotrophic Lateral Sclerosis associated FUS mutation shortens mitochondria and induces neurotoxicity.Scientific reports · 2018Article
- Energy metabolism in ALS: an underappreciated opportunity?Acta neuropathologica · 2018Review
- Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice.Human molecular genetics · 2018Article
- Neurodegeneration-associated FUS is a novel regulator of circadian gene expression.Translational neurodegeneration · 2018Article
- Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions.Neural plasticity · 2018Review
- Mitochondrial quality control in amyotrophic lateral sclerosis: towards a common pathway?Neural regeneration research · 2017Review
- Role and Therapeutic Potential of Astrocytes in Amyotrophic Lateral Sclerosis.Current pharmaceutical design · 2017Review
- Increased Ubqln2 expression causes neuron death in transgenic rats.Journal of neurochemistry · 2016Article
- Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis.Brain research · 2016Review
- Pathogenesis of FUS-associated ALS and FTD: insights from rodent models.Acta neuropathologica communications · 2016Review
- ALS/FTD-associated FUS activates GSK-3β to disrupt the VAPB-PTPIP51 interaction and ER-mitochondria associations.EMBO reports · 2016Article
- Advances in the Development of Disease-Modifying Treatments for Amyotrophic Lateral Sclerosis.CNS drugs · 2016Review
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
Abstract
Ubiquitin-positive inclusion containing Fused in Sarcoma (FUS) defines a new subtype of frontotemporal lobar degeneration (FTLD). FTLD is characterized by progressive alteration in cognitions and it preferentially affects the superficial layers of frontotemporal cortex. Mutation of FUS is linked to amyotrophic lateral sclerosis and to motor neuron disease with FTLD. To examine FUS pathology in FTLD, we developed the first mammalian animal model expressing human FUS with pathogenic mutation and developing progressive loss of memory. In FUS transgenic rats, ubiquitin aggregation and FUS mislocalization were developed primarily in the entorhinal cortex of temporal lobe, particularly in the superficial layers of affected cortex. Overexpression of mutant FUS led to Golgi fragmentation and mitochondrion aggregation. Intriguingly, aggregated ubiquitin was not colocalized with either fragmented Golgi or aggregated mitochondria, and neurons with ubiquitin aggregates were deprived of endogenous TDP-43. Agonists of peroxisome proliferator-activated receptor gamma (PPAR-γ) possess anti-glial inflammation effects and are also shown to preserve the dendrite and dendritic spines of cortical neurons in culture. Here we show that rosiglitazone, a PPAR-γ agonist, rescued the dendrites and dendritic spines of neurons from FUS toxicity and preserved rats' spatial memory. Our FUS transgenic rats would be useful to the mechanistic study of cortical dementia in FTLD. As rosiglitazone is clinically used to treat diabetes, our results would encourage immediate application of PPAR-γ agonists in treating patients with cortical dementia.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.