Henry L Paulson Selected Research

Ataxin-3

2/2021	Antisense Oligonucleotide Therapy Targeted Against ATXN3 Improves Potassium Channel-Mediated Purkinje Neuron Dysfunction in Spinocerebellar Ataxia Type 3.
10/2020	Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3.
1/2020	Druggable genome screen identifies new regulators of the abundance and toxicity of ATXN3, the Spinocerebellar Ataxia type 3 disease protein.
12/2019	Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3.
1/2017	Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3.
1/2015	Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells.
1/2015	Allosteric regulation of deubiquitylase activity through ubiquitination.
10/2013	Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice.
1/2013	The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease.
1/2013	Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3.

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Henry L Paulson Research Topics

Disease

47	Neurodegenerative Diseases (Neurodegenerative Disease) 07/2023 - 07/2002
29	Machado-Joseph Disease (Spinocerebellar Ataxia Type 3) 01/2022 - 06/2003
15	Spinocerebellar Ataxias (Spinocerebellar Ataxia) 10/2023 - 05/2004
13	Alzheimer Disease (Alzheimer's Disease) 01/2024 - 01/2004
11	Huntington Disease (Huntington's Disease) 01/2023 - 08/2004
10	Ataxia (Dyssynergia) 01/2022 - 06/2005
5	Tauopathies 01/2023 - 10/2012
4	Dementia (Dementias) 02/2024 - 08/2002
4	Proteostasis Deficiencies 01/2022 - 05/2004
4	Fragile X Tremor Ataxia Syndrome 08/2015 - 03/2010
3	Parkinson Disease (Parkinson's Disease) 01/2023 - 01/2011
3	Dystonia musculorum deformans type 1 11/2005 - 06/2003
3	Dystonia (Limb Dystonia) 11/2005 - 06/2003
2	Disorders of Excessive Somnolence (Hypersomnia) 06/2024 - 02/2024
2	Frontotemporal Dementia (Semantic Dementia) 10/2018 - 06/2003
2	Myotonic Dystrophy (Dystrophia Myotonica) 01/2014 - 03/2010
2	Gliosis 11/2012 - 10/2010
1	Pathologic Processes 01/2024
1	Cognitive Dysfunction 12/2023
1	Obesity 09/2023
1	Overweight 09/2023
1	Stroke (Strokes) 09/2023
1	Synucleinopathies 01/2023
1	Progressive Supranuclear Palsy (Steele Richardson Olszewski Syndrome) 01/2022
1	Demyelinating Diseases (Demyelinating Disease) 10/2020
1	Progressive Myoclonic Epilepsies (Progressive Myoclonic Epilepsy) 01/2020
1	Muscular Atrophy (Muscle Atrophy) 01/2020
1	Frontotemporal Dementia With Motor Neuron Disease 01/2020
1	Body Weight (Weight, Body) 11/2019
1	Niemann-Pick Diseases (Niemann Pick Disease) 11/2019
1	Type C Niemann-Pick Disease (Niemann Pick Disease, Type C) 11/2019
1	Vascular Dementia (Subcortical Arteriosclerotic Encephalopathy) 01/2019
1	Neuroblastoma 01/2019
1	Infarction (Infarctions) 01/2019
1	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) 10/2018
1	Disease Progression 01/2013
1	Neoplasms (Cancer) 01/2013
1	Motor Neuron Disease (Primary Lateral Sclerosis) 11/2012

Drug/Important Bio-Agent (IBA)

46	Proteins (Proteins, Gene)FDA Link 01/2023 - 07/2002
44	polyglutamineIBA 01/2022 - 07/2002
19	Ataxin-3IBA 02/2021 - 01/2004
15	UbiquitinIBA 01/2022 - 04/2005
8	Deubiquitinating EnzymesIBA 12/2019 - 10/2007
7	Amyloid (Amyloid Fibrils)IBA 01/2024 - 01/2004
5	Proteasome Endopeptidase Complex (Proteasome)IBA 10/2015 - 08/2010
4	tau Proteins (tau Protein)IBA 01/2023 - 06/2013
4	Glutamine (L-Glutamine)FDA Link 02/2021 - 05/2004
4	RNA (Ribonucleic Acid)IBA 08/2015 - 08/2007
3	LipidsIBA 01/2021 - 01/2019
3	5' Untranslated Regions (5' UTR)IBA 08/2015 - 12/2010
3	Ataxin-1IBA 10/2013 - 07/2002
2	Biomarkers (Surrogate Marker)IBA 12/2023 - 01/2022
2	AntibodiesIBA 07/2023 - 01/2019
2	Antisense OligonucleotidesIBA 02/2021 - 01/2019
2	Apolipoproteins E (ApoE)IBA 01/2021 - 11/2014
2	Peptides (Polypeptides)IBA 01/2021 - 01/2019
2	Protein Isoforms (Isoforms)IBA 12/2019 - 09/2011
2	Cysteine Proteases (Cysteine Protease)IBA 01/2015 - 08/2010
2	Messenger RNA (mRNA)IBA 12/2010 - 03/2010
2	NucleotidesIBA 11/2005 - 06/2003
2	Small Interfering RNA (siRNA)IBA 06/2003 - 06/2003
1	aducanumabIBA 01/2024
1	factor AIBA 10/2023
1	CadmiumIBA 09/2023
1	Heavy MetalsIBA 09/2023
1	AntigensIBA 07/2023
1	Amyloidogenic ProteinsIBA 01/2023
1	SynucleinsIBA 01/2023
1	Serine (L-Serine)FDA Link 01/2023
1	Single-Domain AntibodiesIBA 01/2023
1	DNA-Binding Proteins (DNA Binding Protein)IBA 01/2022
1	Superoxide Dismutase-1IBA 01/2022
1	Potassium Channels (Potassium Channel)IBA 02/2021
1	Inositol (Myoinositol)IBA 10/2020
1	Choline (Choline Chloride)IBA 10/2020
1	N-acetylaspartate (N-acetyl aspartate)IBA 10/2020
1	Phospholipids (Phosphatides)FDA LinkGeneric 10/2020
1	BilirubinIBA 11/2019
1	CholesterolIBA 11/2019
1	HDL LipoproteinsIBA 11/2019
1	Selective Serotonin Reuptake Inhibitors (Serotonin Reuptake Inhibitors)IBA 05/2019
1	Citalopram FDA LinkGeneric 05/2019
1	Potassium IonophoresIBA 01/2019
1	Phosphotransferases (Kinase)IBA 01/2019
1	ValinomycinIBA 01/2019
1	Oxytocin Receptors (Oxytocin Receptor)IBA 01/2019
1	Protein AggregatesIBA 01/2019
1	Ligases (Synthetase)IBA 01/2019
1	AntioxidantsIBA 01/2019
1	Carrier Proteins (Binding Protein)IBA 01/2019
1	Ubiquitin-Conjugating EnzymesIBA 01/2018
1	OligonucleotidesIBA 01/2018
1	Aripiprazole (Abilify)FDA Link 11/2016
1	Membrane Glycoproteins (Membrane Glycoprotein)IBA 03/2014
1	3' Untranslated Regions (3' UTR)IBA 01/2014
1	ubiquitin isopeptidaseIBA 01/2013

Therapy/Procedure

18	Therapeutics 06/2024 - 06/2003
2	RNAi Therapeutics 10/2013 - 07/2007
1	Activities of Daily Living (ADL) 02/2024
1	Terminal Care (Care, Terminal) 09/2020