John J Hopwood Selected Research

Mucopolysaccharidosis III (Sanfilippo Syndrome)

1/2019	Synthetic Disaccharide Standards Enable Quantitative Analysis of Stored Heparan Sulfate in MPS IIIA Murine Brain Regions.
7/2018	Lysosomal N-acetyltransferase interacts with ALIX and is detected in extracellular vesicles.
1/2018	Neuronal-specific impairment of heparan sulfate degradation in Drosophila reveals pathogenic mechanisms for Mucopolysaccharidosis type IIIA.
1/2017	A novel conditional Sgsh knockout mouse model recapitulates phenotypic and neuropathic deficits of Sanfilippo syndrome.
1/2017	Axonal dystrophy in the brain of mice with Sanfilippo syndrome.
1/2017	Slow, continuous enzyme replacement via spinal CSF in dogs with the paediatric-onset neurodegenerative disease, MPS IIIA.
5/2016	Low-dose, continuous enzyme replacement therapy ameliorates brain pathology in the neurodegenerative lysosomal disorder mucopolysaccharidosis type IIIA.
5/2016	A Preclinical Study Evaluating AAVrh10-Based Gene Therapy for Sanfilippo Syndrome.
4/2016	Low-dose, continual enzyme delivery ameliorates some aspects of established brain disease in a mouse model of a childhood-onset neurodegenerative disorder.
1/2016	Continual Low-Dose Infusion of Sulfamidase Is Superior to Intermittent High-Dose Delivery in Ameliorating Neuropathology in the MPS IIIA Mouse Brain.

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John J Hopwood Research Topics

Disease

44	Mucopolysaccharidosis III (Sanfilippo Syndrome) 01/2019 - 02/2002
21	Mucopolysaccharidosis VI (Syndrome, Maroteaux-Lamy) 12/2018 - 04/2002
20	Mucopolysaccharidoses 01/2019 - 03/2003
13	Gaucher Disease (Gaucher's Disease) 03/2017 - 01/2002
13	Mucopolysaccharidosis I (Hurler Syndrome) 01/2014 - 09/2002
12	Lysosomal Storage Diseases (Lysosomal Storage Disease) 06/2022 - 01/2004
9	Glycogen Storage Disease Type II (Pompe's Disease) 10/2016 - 01/2002
6	Gliosis 01/2017 - 08/2004
5	Fabry Disease (Fabry's Disease) 06/2011 - 10/2004
5	Mucolipidoses (Sialidosis) 06/2011 - 10/2004
4	Neurodegenerative Diseases (Neurodegenerative Disease) 01/2021 - 04/2016
4	Multiple Sulfatase Deficiency Disease (Mucosulfatidosis) 06/2011 - 03/2003
4	Mucopolysaccharidosis II (Hunter Syndrome) 07/2010 - 03/2003
4	alpha-Mannosidosis 09/2006 - 03/2002
3	Fucosidosis 11/2015 - 10/2005
3	Metachromatic Leukodystrophy (Sulfatide Lipidosis) 06/2011 - 08/2006
3	Disease Progression 07/2010 - 05/2004
3	Hypersensitivity (Allergy) 05/2004 - 03/2003
2	Tay-Sachs Disease 06/2022 - 10/2004
2	Alzheimer Disease (Alzheimer's Disease) 01/2021 - 01/2018
2	Neuroinflammatory Diseases 01/2016 - 05/2015
2	Cognitive Dysfunction 11/2015 - 12/2009
2	Metabolic Diseases (Metabolic Disease) 10/2011 - 08/2004
2	Joint Diseases (Joint Disease) 08/2007 - 04/2006
2	Mucopolysaccharidosis IV (Morquio Syndrome) 02/2007 - 10/2004
2	Central Nervous System Diseases (CNS Diseases) 08/2006 - 01/2005
2	GM1 Gangliosidosis (Gangliosidosis GM1) 10/2005 - 11/2004
2	Neuraminidase deficiency with beta-galactosidase deficiency 10/2005 - 11/2004
2	Neuraminidase 1 deficiency 10/2005 - 11/2004
2	Body Weight (Weight, Body) 04/2005 - 05/2004
1	Sandhoff Disease (Sandhoff's Disease) 06/2022
1	Parkinson Disease (Parkinson's Disease) 03/2017
1	Nervous System Diseases (Neurological Disorders) 05/2016
1	Brain Diseases (Brain Disorder) 04/2016
1	Infections 01/2016

Drug/Important Bio-Agent (IBA)

45	EnzymesIBA 12/2018 - 01/2002
32	N-sulfoglucosamine sulfohydrolase (sulfamidase)IBA 01/2018 - 02/2002
24	Heparitin Sulfate (Heparan Sulfate)IBA 01/2019 - 02/2002
17	N-Acetylgalactosamine-4-Sulfatase (Arylsulfatase B)IBA 12/2018 - 04/2002
15	Proteins (Proteins, Gene)FDA Link 07/2018 - 04/2002
13	OligosaccharidesIBA 05/2016 - 01/2002
11	GlycosaminoglycansIBA 01/2019 - 03/2003
11	Iduronidase (alpha-L-Iduronidase)IBA 01/2014 - 09/2002
7	Biomarkers (Surrogate Marker)IBA 01/2017 - 01/2002
6	Glucosylceramides (Glucocerebrosides)IBA 01/2017 - 01/2002
6	HydrolasesIBA 02/2007 - 05/2003
5	LipidsIBA 01/2021 - 05/2008
5	GlycogenIBA 10/2016 - 01/2005
5	GlycosphingolipidsIBA 01/2016 - 10/2004
5	GlycolipidsIBA 05/2015 - 01/2002
5	Lysergic Acid Diethylamide (LSD)IBA 05/2010 - 08/2004
4	DisaccharidesIBA 01/2019 - 03/2003
4	Glucosylceramidase (Glucocerebrosidase)IBA 01/2017 - 01/2002
4	alpha-Glucosidases (Acid Maltase)IBA 08/2006 - 01/2005
3	lysosomal proteinsIBA 01/2021 - 01/2002
3	Biological ProductsIBA 11/2018 - 01/2002
3	UbiquitinIBA 01/2017 - 08/2004
3	Nonsense Codon (Nonsense Mutation)IBA 01/2014 - 04/2004
3	SulfatasesIBA 05/2012 - 03/2003
3	AcidsIBA 02/2012 - 01/2005
3	Dermatan SulfateIBA 07/2008 - 11/2002
2	Amyloid (Amyloid Fibrils)IBA 06/2022 - 01/2018
2	SynucleinsIBA 01/2021 - 03/2017
2	Polysaccharides (Glycans)IBA 01/2019 - 07/2011
2	Lysosomal Membrane ProteinsIBA 01/2017 - 01/2016
2	GlucosidasesIBA 10/2016 - 02/2012
2	Pharmaceutical PreparationsIBA 10/2016 - 05/2004
2	GangliosidesIBA 04/2016 - 08/2011
2	G(M3) GangliosideIBA 01/2016 - 05/2008
2	Neutralizing AntibodiesIBA 03/2015 - 10/2003
2	CholesterolIBA 08/2011 - 04/2008
2	AntibodiesIBA 12/2009 - 05/2003
2	SphingolipidsIBA 08/2008 - 12/2007
2	beta-GlucosidaseIBA 04/2008 - 11/2005
2	alpha-Mannosidase (LAMAN)IBA 09/2006 - 03/2002
2	Glucose (Dextrose)FDA LinkGeneric 08/2006 - 01/2002
2	alpha-Galactosidase (Beano)IBA 04/2005 - 11/2004
2	TrisaccharidesIBA 01/2005 - 05/2004
2	SaposinsIBA 11/2004 - 01/2002
1	Hexosaminidases (Hexosaminidase)IBA 06/2022
1	Dopamine (Intropin)FDA LinkGeneric 01/2021
1	Acetyl Coenzyme A (Acetyl-CoA)IBA 07/2018
1	AcetyltransferasesIBA 07/2018
1	Choline O-Acetyltransferase (Choline Acetyltransferase)IBA 01/2017
1	Detergents (Detergent)IBA 01/2017
1	Glutamic Acid (Glutamate)FDA Link 01/2017
1	Tyrosine 3-Monooxygenase (Tyrosine Hydroxylase)IBA 01/2017
1	Cholinergic Agents (Cholinergics)IBA 01/2017
1	sphingosyl beta-glucosideIBA 01/2017
1	Glial Fibrillary Acidic ProteinIBA 01/2016
1	LectinsIBA 01/2016
1	Filipin (Filipin III)IBA 01/2016

Therapy/Procedure

23	Enzyme Replacement Therapy 05/2016 - 09/2002
20	Therapeutics 07/2018 - 01/2002
4	Cannula 01/2017 - 01/2016
4	Bone Marrow Transplantation (Transplantation, Bone Marrow) 01/2013 - 10/2003
3	Injections 01/2016 - 07/2004
2	Spinal Injections 01/2015 - 02/2010
1	Spinal Infusions 01/2017
1	Infusion Pumps (Infusion Pump) 05/2016