Jonathan D Cooper Selected Research

Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis)

1/2019	Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis.
1/2017	Synergistic effects of treating the spinal cord and brain in CLN1 disease.
1/2017	mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases.
12/2016	Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3).
1/2016	Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis.
9/2015	Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.
2/2015	Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis.
9/2014	An anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosis.
3/2014	Partial correction of the CNS lysosomal storage defect in a mouse model of juvenile neuronal ceroid lipofuscinosis by neonatal CNS administration of an adeno-associated virus serotype rh.10 vector expressing the human CLN3 gene.
11/2013	Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease).

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Jonathan D Cooper Research Topics

Disease

33	Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis) 01/2019 - 06/2002
12	Neurodegenerative Diseases (Neurodegenerative Disease) 01/2017 - 06/2002
8	Lysosomal Storage Diseases (Lysosomal Storage Disease) 01/2022 - 04/2008
7	Neuroinflammatory Diseases 01/2022 - 06/2010
7	Gliosis 01/2022 - 01/2010
6	Mucopolysaccharidosis III (Sanfilippo Syndrome) 01/2022 - 01/2018
3	Schizophrenia (Dementia Praecox) 11/2022 - 05/2012
3	Mucopolysaccharidosis I (Hurler Syndrome) 11/2020 - 01/2015
3	Alzheimer Disease (Alzheimer's Disease) 09/2014 - 11/2003
2	Atrophy 01/2022 - 09/2014
2	Seizures (Absence Seizure) 01/2022 - 01/2016
2	Hepatomegaly 01/2022 - 01/2018
2	Mucopolysaccharidoses 01/2021 - 01/2018
2	Retinal Degeneration 01/2018 - 05/2006
2	Disease Progression 10/2012 - 02/2007
2	Type C Niemann-Pick Disease (Niemann Pick Disease, Type C) 03/2012 - 06/2011
2	Gaucher Disease (Gaucher's Disease) 04/2011 - 02/2008
2	Parkinson Disease (Parkinson's Disease) 09/2010 - 01/2010
2	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) 10/2004 - 06/2004
1	Psychotic Disorders (Schizoaffective Disorder) 11/2022
1	Cognitive Dysfunction 01/2022
1	Central Nervous System Diseases (CNS Diseases) 09/2018
1	Bone Diseases (Bone Disease) 01/2018
1	Inflammation (Inflammations) 01/2018
1	Sandhoff Disease (Sandhoff's Disease) 01/2018
1	Splenomegaly 01/2018
1	Phenylketonurias (Phenylketonuria) 01/2017
1	Mental Disorders (Mental Disorder) 12/2015
1	Encephalitis (Encephalitis, Rasmussen) 11/2015
1	Lymphoma (Lymphomas) 01/2015
1	Traumatic Brain Injuries (Traumatic Brain Injury) 09/2014
1	Epilepsy (Aura) 05/2013
1	Muscular Atrophy (Muscle Atrophy) 02/2013
1	Bipolar Disorder (Manic Depressive Psychosis) 05/2012

Drug/Important Bio-Agent (IBA)

17	EnzymesIBA 01/2020 - 03/2006
15	Proteins (Proteins, Gene)FDA Link 01/2021 - 07/2004
4	alpha-N-acetyl-D-glucosaminidase (alpha-N-acetylglucosaminidase)IBA 01/2021 - 01/2018
4	palmitoyl-protein thioesteraseIBA 01/2017 - 01/2007
3	Heparitin Sulfate (Heparan Sulfate)IBA 01/2021 - 09/2018
3	Tripeptidyl-Peptidase 1IBA 01/2016 - 04/2008
3	AutoantibodiesIBA 01/2011 - 06/2002
3	Peptides (Polypeptides)IBA 06/2010 - 11/2003
2	N-Methyl-D-Aspartate Receptors (NMDA Receptors)IBA 11/2022 - 10/2012
2	GlycosaminoglycansIBA 01/2022 - 01/2015
2	Hexosaminidases (Hexosaminidase)IBA 01/2021 - 01/2018
2	Acetylglucosaminidase (NAGase)IBA 01/2021 - 09/2018
2	Iduronidase (alpha-L-Iduronidase)IBA 03/2018 - 01/2015
2	TrehaloseIBA 01/2018 - 01/2017
2	Glutamic Acid (Glutamate)FDA Link 12/2015 - 06/2002
2	Antipsychotic Agents (Antipsychotics)IBA 11/2015 - 05/2012
2	CytokinesIBA 09/2014 - 01/2010
2	Lysergic Acid Diethylamide (LSD)IBA 09/2014 - 02/2008
2	Therapeutic UsesIBA 03/2014 - 10/2012
2	Membrane Proteins (Integral Membrane Proteins)IBA 11/2013 - 01/2012
2	Pharmaceutical PreparationsIBA 05/2013 - 01/2011
2	CholesterolIBA 03/2012 - 06/2011
2	Cathepsin DIBA 12/2011 - 01/2008
2	Amyloid (Amyloid Fibrils)IBA 06/2011 - 01/2010
2	AMPA Receptors (AMPA Receptor)IBA 02/2011 - 06/2008
2	Immunoglobulin G (IgG)IBA 01/2011 - 02/2007
2	Peptide Hydrolases (Proteases)FDA Link 04/2008 - 01/2008
2	AntigensIBA 02/2007 - 10/2004
1	Ketamine FDA LinkGeneric 11/2022
1	CannabidiolIBA 01/2022
1	N-acetylglucosamine-6-sulfataseIBA 12/2021
1	LipidsIBA 11/2020
1	CD68 MoleculeIBA 01/2018
1	Carrier Proteins (Binding Protein)IBA 01/2018
1	Glial Fibrillary Acidic ProteinIBA 01/2018
1	Glyceraldehyde-3-Phosphate Dehydrogenases (GAPD)IBA 01/2018
1	Guanine NucleotidesIBA 01/2018
1	HydrolasesIBA 01/2018
1	Microtubule-Associated Proteins (Microtubule-Associated Protein 2)IBA 01/2018
1	Phosphates (Orthophosphate)IBA 01/2018
1	Transcription Factors (Transcription Factor)IBA 01/2018
1	Protein Kinase C-alphaIBA 01/2018
1	pegvaliaseIBA 01/2017
1	Phenylalanine Ammonia-LyaseIBA 01/2017
1	Adenosine Monophosphate (AMP)IBA 12/2016
1	Type 4 Cyclic Nucleotide PhosphodiesterasesIBA 12/2016
1	mitochondrial ATPase subunit cIBA 01/2016
1	N-acetylaspartate (N-acetyl aspartate)IBA 12/2015
1	Glutathione (Reduced Glutathione)IBA 12/2015
1	Poly I-CIBA 12/2015
1	Protons (Proton)IBA 12/2015
1	2- (4- (4- methyl- 6- phenylpyridazin- 3- yl)piperazin- 1- yl)pyrimidineIBA 09/2014
1	Kainic Acid (Kainate)IBA 09/2014
1	Lysosomal Membrane ProteinsIBA 03/2014
1	Cathepsin FIBA 04/2013
1	Memantine (Namenda)FDA Link 10/2012
1	LithiumIBA 05/2012
1	GlycosphingolipidsIBA 03/2012

Therapy/Procedure

7	Therapeutics 01/2022 - 06/2008
7	Enzyme Replacement Therapy 01/2022 - 04/2008
1	Intravenous Administration 01/2018
1	Hematopoietic Stem Cell Transplantation 01/2018
1	Traction 09/2015
1	Stem Cell Transplantation 01/2015
1	Denervation 02/2013
1	Bone Marrow Transplantation (Transplantation, Bone Marrow) 06/2012