Dr. Henry Paulson, MD

• Neurology

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• Yale University

  Medical School

• Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study, 2013-11-13

• The S-Factor, a New Measure of Disease Severity in Spinocerebellar Ataxia: Findings and Implications, 2022-08-12

• Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1, 2013-10-02

• Druggable genome screen identifies new regulators of the abundance and toxicity of ATXN3, the Spinocerebellar Ataxia Type 3 disease protein, 2019-11-26

• The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease, 2013-06-18

• Spinocerebellar ataxias: prospects and challenges for therapy development, 2019-04-11

• Establishment of a Novel Fluorescence-Based Method to Evaluate Chaperone-Mediated Autophagy in a Single Neuron, 2012-02-07

• The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's Disease protein, huntingtin, 2017-10-03

• Alzheimer's Disease and Environmental Exposure to Lead: The Epidemiologic Evidence and Potential Role of Epigenetics, 2013-01-29

• The E3 Ubiquitin Ligase CHIP and the Molecular Chaperone Hsc70 Form a Dynamic, Tethered Complex, 2013-08-02

• Associations of healthy lifestyle and socioeconomic status with cognitive function in U.S. older adults, 2023-05-09

• DistinctC9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity, 2016-10-24

• Polyglutamine spinocerebellar ataxias - from genes to potential treatments, 2017-08-17

• Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3, 2020-10-21

• Antisense oligonucleotide therapy rescues aggresome formation in a novel Spinocerebellar Ataxia type 3 human embryonic stem cell line., 2019-07-16

• Oligonucleotide therapy mitigates disease in Spinocerebellar Ataxia Type 3 mice, 2018-08-06

• Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease, Spinocerebellar Ataxia type 3, 2011-09-07

• Understanding the Role of the Josephin Domain in the PolyUb Binding and Cleavage Properties of Ataxin-3, 2010-08-26

• Ubiquilin-2 regulates pathological alpha-synuclein, 2023-01-06

• Splice isoform-specific suppression of the CaV2.1 variant underlying Spinocerebellar ataxia type 6, 2011-04-29

• Ataxin-3 Plays a Role in Mouse Myogenic Differentiation through Regulation of Integrin Subunit Levels, 2010-07-23

• Alterations in cerebellar physiology are associated with a stiff-legged gait inAtcayji-hesmice, 2014-04-12

• Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties, 2010-10-27

• Rational affinity maturation of anti-amyloid antibodies with high conformational and sequence specificity, 2021-03-04

• Citalopram reduces aggregation of ATXN3 in a YAC transgenic mouse model of Machado-Joseph disease, 2018-09-04

• Genetics of Dementia, 2012-01-21

• Antisense oligonucleotide therapy targeted againstATXN3improves potassium channel-mediated Purkinje neuron dysfunction in spinocerebellar ataxia type 3, 2020-08-10

• CGG Repeat Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome, 2013-04-18

• Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3, 2015-06-30

• Pathogenesis of SCA3 and implications for other polyglutamine diseases, 2019-10-24

• Genetic meta-analysis of diagnosed Alzheimer's disease identifies new risk loci and implicates Aß, tau, immunity and lipid processing, 2019-02-28

• Upregulation of the ESCRT pathway and multivesicular bodies accelerates degradation of proteins associated with neurodegeneration, 2023-01-22

• Genome-Wide DNA Methylation Differences Between Late-Onset Alzheimer's Disease and Cognitively Normal Controls in Human Frontal Cortex, 2013-04-02

• In vivomolecular signatures of cerebellar pathology in spinocerebellar ataxia type 3, 2020-07-04

• In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70 interacting protein (CHIP) supports an aggregation model of pathogenesis, 2008-11-08

• Polyglutamine Neurodegeneration: Protein Misfolding Revisited, 2008-09-06

• Histone Deacetylases Suppress CGG Repeat-Induced Neurodegeneration Via Transcriptional Silencing in Models of Fragile X Tremor Ataxia Syndrome, 2010-12-09

• Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3, 2022-10-05

• RTL8 promotes nuclear localization of UBQLN2 to subnuclear compartments associated with protein quality control, 2022-03-05

• Analysis of the Tau-Associated Proteome Reveals that Exchange of Hsp70 for Hsp90 Is Involved in Tau Degradation, 2012-07-25

• Cognitive Decline and Aging: The role of Concussive and Subconcussive Impacts, 2013-04-02

• Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study, 2012-06-27

• Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways, 2018-09-19

• Modeling UBQLN2-mediated neurodegenerative disease in mice: shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity, 2020-07-10

• COMPASS: A computational model to predict changes in MMSE scores 24-months after initial assessment of Alzheimer's disease, 2016-10-05

• The ubiquitin ligase FBG1 promotes the degradation of the disease-linked protein torsinA through the ubiquitin proteasome pathway and macroautophagy, 2012-08-20

• Caregiver-Reported Barriers to Quality End-of-Life Care in Dementia with Lewy Bodies: A Qualitative Analysis, 2020-01-06

• Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP, 2011-08-19

• Shared and divergent phase separation and aggregation properties of brain-expressed ubiquilins, 2021-01-11

• Regional and age-dependent changes in ubiquitination in cellular and mouse models of spinocerebellar ataxia type 3, 2023-04-14

• Quantitative flow cytometric selection of tau conformational nanobodies specific for pathological aggregates, 2023-08-09