Search Results - "Paulson, L."
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Pathogenesis of SCA3 and implications for other polyglutamine diseases
Published in Neurobiology of disease (01-02-2020)“…Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine (polyQ) diseases, caused by CAG repeat expansions in the coding regions…”
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CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome
Published in Neuron (Cambridge, Mass.) (08-05-2013)“…Fragile X-associated tremor ataxia syndrome (FXTAS) results from a CGG repeat expansion in the 5′ UTR of FMR1. This repeat is thought to elicit toxicity as…”
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Multi-sensory Gamma Stimulation Ameliorates Alzheimer’s-Associated Pathology and Improves Cognition
Published in Cell (04-04-2019)“…We previously reported that inducing gamma oscillations with a non-invasive light flicker (gamma entrainment using sensory stimulus or GENUS) impacted…”
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Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics
Published in Current Alzheimer research (01-06-2012)“…Several lines of evidence indicate that the etiology of late-onset Alzheimer's disease (LOAD) is complex, with significant contributions from both genes and…”
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RNA-mediated neurodegeneration in repeat expansion disorders
Published in Annals of neurology (01-03-2010)“…Most neurodegenerative disorders are thought to result primarily from the accumulation of misfolded proteins, which interfere with protein homeostasis in…”
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Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice
Published in Annals of neurology (01-07-2018)“…Objective Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease, is the most common dominantly inherited ataxia. Despite advances in…”
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Polyglutamine neurodegeneration: protein misfolding revisited
Published in Trends in neurosciences (Regular ed.) (01-10-2008)“…Polyglutamine diseases are a major cause of neurodegeneration worldwide. Recent studies highlight the importance of protein quality control mechanisms in…”
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Ube2w and Ataxin-3 Coordinately Regulate the Ubiquitin Ligase CHIP
Published in Molecular cell (19-08-2011)“…The mechanisms by which ubiquitin ligases are regulated remain poorly understood. Here we describe a series of molecular events that coordinately regulate…”
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Peri-Infarct Upregulation of the Oxytocin Receptor in Vascular Dementia
Published in Journal of neuropathology and experimental neurology (01-05-2019)“…Abstract Vascular dementia (VaD) is cognitive decline linked to reduced cerebral blood perfusion, yet there are few therapeutic options to protect cognitive…”
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Alzheimer’s pathology causes impaired inhibitory connections and reactivation of spatial codes during spatial navigation
Published in Cell reports (Cambridge) (20-04-2021)“…Synapse loss and altered synaptic strength are thought to underlie cognitive impairment in Alzheimer’s disease (AD) by disrupting neural activity essential for…”
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Associations of healthy lifestyle and socioeconomic status with cognitive function in U.S. older adults
Published in Scientific reports (09-05-2023)“…We investigated the complex relations of socioeconomic status (SES) and healthy lifestyles with cognitive functions among older adults in 1313 participants,…”
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RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome
Published in Human molecular genetics (01-08-2015)“…Fragile X-associated tremor ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by a CGG trinucleotide repeat expansion in the 5' UTR of the Fragile…”
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Accelerated neurodegeneration through chaperone-mediated oligomerization of tau
Published in The Journal of clinical investigation (01-10-2013)“…Aggregation of tau protein in the brain is associated with a class of neurodegenerative diseases known as tauopathies. FK506 binding protein 51 kDa (FKBP51,…”
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Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3
Published in The Journal of neuroscience (07-09-2011)“…The relationship between cerebellar dysfunction, motor symptoms, and neuronal loss in the inherited ataxias, including the polyglutamine disease…”
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Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways
Published in Journal of pharmacological sciences (01-07-2023)“…Missense mutations of ubiquilin 2 (UBQLN2) have been identified to cause X-linked amyotrophic lateral sclerosis (ALS). Proteasome-mediated protein degradation…”
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Estimating pathogen‐spillover risk using host–ectoparasite interactions
Published in Ecology and evolution (01-06-2024)“…Pathogen spillover corresponds to the transmission of a pathogen or parasite from an original host species to a novel host species, preluding disease…”
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Genome-wide DNA methylation differences between late-onset Alzheimer's disease and cognitively normal controls in human frontal cortex
Published in Journal of Alzheimer's disease (01-01-2012)“…Evidence supports a role for epigenetic mechanisms in the pathogenesis of late-onset Alzheimer's disease (LOAD), but little has been done on a genome-wide…”
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Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly
Published in Proceedings of the National Academy of Sciences - PNAS (30-10-2018)“…UBQLN2 is one of a family of proteins implicated in ubiquitin-dependent protein quality control and integrally tied to human neurodegenerative disease. Whereas…”
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siRNA-mediated gene silencing in vitro and in vivo
Published in Nature biotechnology (01-10-2002)“…RNA interference is now established as an important biological strategy for gene silencing, but its application to mammalian cells has been limited by…”
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Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models
Published in Molecular therapy. Nucleic acids (16-06-2017)“…The most common dominantly inherited ataxia, spinocerebellar ataxia type 3 (SCA3), is an incurable neurodegenerative disorder caused by a CAG repeat expansion…”
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