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List of works by Kim Hemsley

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Functional correction of CNS lesions in an MPS-IIIA mouse model by intracerebral AAV-mediated delivery of sulfamidase and SUMF1 genes

scientific article published on 27 August 2007
Characterization of a C57BL/6 congenic mouse strain of mucopolysaccharidosis type IIIA.

scientific article published on 7 July 2006
Injection of recombinant human sulfamidase into the CSF via the cerebellomedullary cistern in MPS IIIA mice.

scientific article published on 12 December 2006
Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice

scientific article published on 25 March 2008
Development of motor deficits in a murine model of mucopolysaccharidosis type IIIA (MPS-IIIA).

scientific article published in March 2005
Examination of intravenous and intra-CSF protein delivery for treatment of neurological disease.

scientific article
Intracerebral injection of sulfamidase delays neuropathology in murine MPS-IIIA.

scientific article
Endo-lysosomal and autophagic dysfunction: a driving factor in Alzheimer's disease?

scientific article published on 27 December 2016
Effect of cisternal sulfamidase delivery in MPS IIIA Huntaway dogs--a proof of principle study.

scientific article published on 28 July 2009
Impact of high-dose, chemically modified sulfamidase on pathology in a murine model of MPS IIIA.

scientific article
Enzyme replacement reduces neuropathology in MPS IIIA dogs

scientific article published on 29 April 2011
Allogeneic stem cell transplantation does not improve neurological deficits in mucopolysaccharidosis type IIIA mice

scientific article published on 27 July 2010
Changes in muscle tone are regulated by D1 and D2 dopamine receptors in the ventral striatum and D1 receptors in the substantia nigra.

scientific article
Lessons learnt from animal models: pathophysiology of neuropathic lysosomal storage disorders.

scientific article published on 07 May 2010
Validation of a heparan sulfate-derived disaccharide as a marker of accumulation in murine mucopolysaccharidosis type IIIA.

scientific article
Treatment of canine fucosidosis by intracisternal enzyme infusion

scientific article published on 06 May 2011
Helper-dependent canine adenovirus vector-mediated transgene expression in a neurodegenerative lysosomal storage disorder

scientific article published on 22 September 2011
Delivery of therapeutic protein for prevention of neurodegenerative changes: comparison of different CSF-delivery methods

scientific article
Axonal dystrophy in the brain of mice with Sanfilippo syndrome

scientific article published on 7 June 2017
A Preclinical Study Evaluating AAVrh10-Based Gene Therapy for Sanfilippo Syndrome

scientific article
SGSH gene transfer in mucopolysaccharidosis type IIIA mice using canine adenovirus vectors

scientific article published on 14 February 2010
Raclopride and chlorpromazine, but not clozapine, increase muscle rigidity in the rat: relationship with D2 dopamine receptor occupancy

scientific article
Evaluation of enzyme dose and dose-frequency in ameliorating substrate accumulation in MPS IIIA Huntaway dog brain

scientific article published on 25 November 2014
Intracisternal enzyme replacement therapy in lysosomal storage diseases: routes of absorption into brain

scientific article published on 01 June 2011
The effects of an irreversible dopamine receptor antagonist, N-ethoxycarbonyl-2-ethoxy-1,2-dihydroquinoline (EEDQ), on the regulation of muscle tone in the rat: the role of the substantia nigra

scientific article published in July 1998
Determination of the role of injection site on the efficacy of intra-CSF enzyme replacement therapy in MPS IIIA mice

scientific article published on 12 March 2015
Neonatal Bone Marrow Transplantation in MPS IIIA Mice

scientific article published on 10 August 2012
Emerging therapies for neurodegenerative lysosomal storage disorders - from concept to reality.

scientific article published on 17 May 2011
Low-dose, continuous enzyme replacement therapy ameliorates brain pathology in the neurodegenerative lysosomal disorder mucopolysaccharidosis type IIIA.

scientific article published on 13 January 2016
Low-dose, continual enzyme delivery ameliorates some aspects of established brain disease in a mouse model of a childhood-onset neurodegenerative disorder

scientific article published on 25 November 2015
Intracisternal enzyme replacement therapy in lysosomal storage diseases: dispersal pathways, regional enzyme concentrations and the effect of posttreatment posture.

scientific article published in October 2013
Reduction in open field activity in the absence of memory deficits in the AppNL-G-F knock-in mouse model of Alzheimer's disease

scientific article published on 5 September 2017
A novel conditional Sgsh knockout mouse model recapitulates phenotypic and neuropathic deficits of Sanfilippo syndrome

scientific article published on 27 April 2017
Embryonic stem cell-derived glial precursors as a vehicle for sulfamidase production in the MPS-IIIA mouse brain.

scientific article published on 26 March 2010
Atropine acts in the ventral striatum to reduce raclopride-induced catalepsy.

scientific article published in July 2001
Behavioural characterisation of the alpha-mannosidosis guinea pig.

scientific article published on 10 August 2007
Disease stage determines the efficacy of treatment of a paediatric neurodegenerative disease

scientific article published in June 2014
Continual Low-Dose Infusion of Sulfamidase Is Superior to Intermittent High-Dose Delivery in Ameliorating Neuropathology in the MPS IIIA Mouse Brain

scientific article published on January 1, 2016
Exocytosis is impaired in mucopolysaccharidosis IIIA mouse chromaffin cells

scientific article published on 25 September 2012
Development of cerebellar pathology in the canine model of mucopolysaccharidosis type IIIA (MPS IIIA).

scientific article published on 22 October 2014
Adeno-associated viral gene therapy for mucopolysaccharidoses exhibiting neurodegeneration

scientific article
Dopamine receptors in the subthalamic nucleus are involved in the regulation of muscle tone in the rat.

scientific article published in January 2002
Survival and engraftment of mouse embryonic stem cell-derived implants in the guinea pig brain

scientific article published on 01 October 2005
Neuronal-specific impairment of heparan sulfate degradation in Drosophila reveals pathogenic mechanisms for Mucopolysaccharidosis type IIIA.

scientific article published in February 2018
Slow, continuous enzyme replacement via spinal CSF in dogs with the paediatric-onset neurodegenerative disease, MPS IIIA.

scientific article published on 10 November 2016
In vitro characterization of genetically modified embryonic stem cells as a therapy for murine mucopolysaccharidosis type IIIA.

scientific article published on February 2004
β-Mannosidosis in German Shepherd Dogs

scientific article published on 14 April 2019
Glycosphingolipid analysis in a naturally occurring ovine model of acute neuronopathic Gaucher disease.

scientific article published on 11 March 2016
Early disease course is unaltered in mucopolysaccharidosis type IIIA (MPS IIIA) mice lacking α-synuclein

scientific article published on 25 April 2019
Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates

scientific article published on 22 October 2015

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