Harry T Orr Selected Research

Spinocerebellar Ataxias (Spinocerebellar Ataxia)

1/2022	Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1.
1/2022	Reduction of mutant ATXN1 rescues premature death in a conditional SCA1 mouse model.
1/2021	Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1.
1/2021	Modulation of ATXN1 S776 phosphorylation reveals the importance of allele-specific targeting in SCA1.
11/2020	Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1.
9/2020	Antisense Oligonucleotide Therapeutic Approach for Suppression of Ataxin-1 Expression: A Safety Assessment.
1/2020	The ataxin-1 interactome reveals direct connection with multiple disrupted nuclear transport pathways.
11/2019	Treadmill training increases the motor activity and neuron survival of the cerebellum in a mouse model of spinocerebellar ataxia type 1.
11/2018	Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles.
1/2018	ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism.

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Harry T Orr Research Topics

Disease

57	Spinocerebellar Ataxias (Spinocerebellar Ataxia) 01/2022 - 01/2002
36	Neurodegenerative Diseases (Neurodegenerative Disease) 01/2022 - 01/2002
6	Ataxia (Dyssynergia) 03/2022 - 03/2010
5	Disease Progression 01/2020 - 01/2002
3	Nervous System Diseases (Neurological Disorders) 06/2022 - 01/2008
3	Cerebellar Diseases (Cerebellar Syndrome) 03/2022 - 06/2009
3	Huntington Disease (Huntington's Disease) 05/2011 - 11/2003
2	Atrophy 08/2015 - 08/2011
1	Memory Disorders (Memory Loss) 01/2021
1	Neoplasms (Cancer) 09/2020
1	Proteostasis Deficiencies 03/2015
1	Dementia (Dementias) 05/2013
1	Cerebellar Ataxia (Dysmetria) 08/2011
1	Leukemia 06/2005
1	Inborn Genetic Diseases (Disease, Hereditary) 08/2002

Drug/Important Bio-Agent (IBA)

48	polyglutamineIBA 01/2022 - 01/2002
38	Ataxin-1IBA 01/2022 - 01/2002
18	Proteins (Proteins, Gene)FDA Link 01/2022 - 01/2002
9	Glutamine (L-Glutamine)FDA Link 11/2018 - 04/2005
5	RNA (Ribonucleic Acid)IBA 11/2018 - 07/2010
5	Mutant Proteins (Protein, Mutant)IBA 03/2015 - 06/2002
3	Glutamic Acid (Glutamate)FDA Link 03/2010 - 10/2004
3	Serine (L-Serine)FDA Link 07/2009 - 05/2003
2	Phosphotransferases (Kinase)IBA 01/2021 - 05/2013
2	Cyclic AMP-Dependent Protein Kinases (cAMP-Dependent Protein Kinase)IBA 01/2018 - 01/2013
2	Biological ProductsIBA 01/2013 - 04/2005
2	Inositol (Myoinositol)IBA 12/2011 - 03/2010
2	Biomarkers (Surrogate Marker)IBA 12/2011 - 03/2010
2	AtaxinsIBA 07/2010 - 10/2004
1	Complement System Proteins (Complement)IBA 06/2022
1	TOR Serine-Threonine KinasesIBA 03/2022
1	Mechanistic Target of Rapamycin Complex 1IBA 03/2022
1	Ion Channels (Ion Channel)IBA 11/2020
1	Amyloid (Amyloid Fibrils)IBA 09/2020
1	Amyloid Precursor Protein Secretases (beta-Secretase)IBA 09/2020
1	Peptide Hydrolases (Proteases)FDA Link 09/2020
1	GuanineIBA 11/2019
1	CytosineIBA 11/2019
1	Adenine FDA LinkGeneric 11/2019
1	Antisense OligonucleotidesIBA 11/2018
1	Potassium Channels (Potassium Channel)IBA 08/2015
1	Large-Conductance Calcium-Activated Potassium Channels (Maxi-K Channels)IBA 08/2015
1	Flufenamic AcidIBA 08/2015
1	Protein Serine-Threonine Kinases (Protein-Serine-Threonine Kinase)IBA 05/2013
1	Protons (Proton)IBA 12/2011
1	Doxycycline (Periostat)FDA LinkGeneric 12/2011
1	Epidermal Growth Factor (EGF)IBA 11/2011
1	Potassium Channel Blockers (Blockers, Potassium Channel)IBA 08/2011
1	AmifampridineIBA 08/2011
1	Shal Potassium ChannelsIBA 08/2011
1	AminopyridinesIBA 08/2011
1	Intercellular Signaling Peptides and Proteins (Growth Factors)IBA 08/2011
1	PotassiumIBA 08/2011
1	Brain-Derived Neurotrophic Factor (BDNF)IBA 08/2011
1	Peptides (Polypeptides)IBA 05/2011
1	N-acetylaspartate (N-acetyl aspartate)IBA 03/2010
1	CalciumIBA 06/2009
1	NucleotidesIBA 03/2009
1	Insulin-Like PeptidesIBA 01/2008
1	Antisense RNA (Anti-Sense RNA)IBA 01/2008
1	Neuroprotective AgentsIBA 05/2007
1	LithiumIBA 05/2007
1	Lithium Carbonate (Eskalith)FDA LinkGeneric 05/2007
1	DNA (Deoxyribonucleic Acid)IBA 10/2004
1	A-factor (Streptomyces)IBA 11/2003
1	Leucine (L-Leucine)FDA Link 09/2003
1	Nuclear Proteins (Protein, Nuclear)IBA 09/2003
1	Phosphatidylinositol 3-Kinase (1 Phosphatidylinositol 3 Kinase)IBA 05/2003
1	EnzymesIBA 06/2002
1	Glycogen Synthase Kinase 3IBA 06/2002
1	Amino Acids FDA Link 01/2002

Therapy/Procedure

11	Therapeutics 06/2022 - 08/2004
1	Mechanical Ventilators (Ventilator) 01/2018
1	Palliative Care (Palliative Therapy) 01/2018
1	Immunotherapy 12/2015