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List of works by Kim Hemsley

A Preclinical Study Evaluating AAVrh10-Based Gene Therapy for Sanfilippo Syndrome

scientific article

A novel conditional Sgsh knockout mouse model recapitulates phenotypic and neuropathic deficits of Sanfilippo syndrome

scientific article published on 27 April 2017

AAVrh10 Vector Corrects Disease Pathology in MPS IIIA Mice and Achieves Widespread Distribution of SGSH in Large Animal Brains

scientific article published on 10 December 2019

Adeno-associated viral gene therapy for mucopolysaccharidoses exhibiting neurodegeneration

scientific article

Allogeneic stem cell transplantation does not improve neurological deficits in mucopolysaccharidosis type IIIA mice

scientific article published on 27 July 2010

Atropine acts in the ventral striatum to reduce raclopride-induced catalepsy.

scientific article published in July 2001

Atropine reduces raclopride-induced muscle rigidity by acting in the ventral region of the striatum

scientific article published in January 2002

Axonal dystrophy in the brain of mice with Sanfilippo syndrome

scientific article published on 7 June 2017

Behavioural characterisation of the alpha-mannosidosis guinea pig.

scientific article published on 10 August 2007

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

Characterization of a C57BL/6 congenic mouse strain of mucopolysaccharidosis type IIIA.

scientific article published on 7 July 2006

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 December 2015

Delivery of therapeutic protein for prevention of neurodegenerative changes: comparison of different CSF-delivery methods

scientific article

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

Development of cerebellar pathology in the canine model of mucopolysaccharidosis type IIIA (MPS IIIA).

scientific article published on 22 October 2014

Development of motor deficits in a murine model of mucopolysaccharidosis type IIIA (MPS-IIIA).

scientific article published in March 2005

Directed differentiation and characterization of genetically modified embryonic stem cells for therapy

scientific article published on January 2006

Disease stage determines the efficacy of treatment of a paediatric neurodegenerative disease

scientific article published in June 2014

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

scientific article published in January 2002

Early disease course is unaltered in mucopolysaccharidosis type IIIA (MPS IIIA) mice lacking α-synuclein

scientific article published on 25 April 2019

Effect of cisternal sulfamidase delivery in MPS IIIA Huntaway dogs--a proof of principle study.

scientific article published on 28 July 2009

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

Emerging therapies for neurodegenerative lysosomal storage disorders - from concept to reality.

scientific article published on 17 May 2011

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

scientific article published on 27 December 2016

Enzyme replacement reduces neuropathology in MPS IIIA dogs

scientific article published on 29 April 2011

Evaluation of Disease Lesions in the Developing Canine MPS IIIA Brain

scientific article published on 20 June 2018

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

scientific article published on 25 November 2014

Examination of intravenous and intra-CSF protein delivery for treatment of neurological disease.

scientific article

Exocytosis is impaired in mucopolysaccharidosis IIIA mouse chromaffin cells

scientific article published on 25 September 2012

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

Genetic manipulation of murine embryonic stem cells with enhanced green fluorescence protein and sulfatase-modifying factor I genes.

scientific article published on May 2010

Glycosphingolipid analysis in a naturally occurring ovine model of acute neuronopathic Gaucher disease.

scientific article published on 11 March 2016

Helper-dependent canine adenovirus vector-mediated transgene expression in a neurodegenerative lysosomal storage disorder

scientific article published on 22 September 2011

Impact of high-dose, chemically modified sulfamidase on pathology in a murine model of MPS IIIA.

scientific article

In vitro characterization of genetically modified embryonic stem cells as a therapy for murine mucopolysaccharidosis type IIIA.

scientific article published on February 2004

Injection of recombinant human sulfamidase into the CSF via the cerebellomedullary cistern in MPS IIIA mice.

scientific article published on 12 December 2006

Intracerebral gene therapy for mucopolysaccharidosis type IIIB syndrome

scientific article published on 13 July 2017

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

scientific article

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

Intracisternal enzyme replacement therapy in lysosomal storage diseases: routes of absorption into brain

scientific article published on 01 June 2011

Is the eye a window to the brain in Sanfilippo syndrome?

scientific article published on 17 November 2020

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

scientific article published on 07 May 2010

Longitudinal Magnetic Resonance Spectroscopy and Diffusion Tensor Imaging in Sheep (Ovis aries) With Quinolinic Acid Lesions of the Striatum: Time-Dependent Recovery of N-Acetylaspartate and Fractional Anisotropy

scientific article published on 02 August 2020

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

Low-dose, continuous enzyme replacement therapy ameliorates brain pathology in the neurodegenerative lysosomal disorder mucopolysaccharidosis type IIIA.

scientific article published on 13 January 2016

Lysosomal Dysregulation in the Murine AppNL-G-F/NL-G-F Model of Alzheimer's Disease

scientific article published on 07 January 2020

MPS-IIIA mice acquire autistic behaviours with age.

scientific article published on 8 March 2018

Neonatal Bone Marrow Transplantation in MPS IIIA Mice

scientific article published on 10 August 2012

Neuronal-specific impairment of heparan sulfate degradation in Drosophila reveals pathogenic mechanisms for Mucopolysaccharidosis type IIIA.

scientific article published in February 2018

Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice

scientific article published on 25 March 2008

Primary culture of neural cells isolated from the cerebellum of newborn and adult mucopolysaccharidosis type IIIA mice

scientific article published on 23 February 2008

Raclopride and chlorpromazine, but not clozapine, increase muscle rigidity in the rat: relationship with D2 dopamine receptor occupancy

scientific article

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

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

scientific article published on 14 February 2010

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

scientific article published on 10 November 2016

Survival and engraftment of mouse embryonic stem cell-derived implants in the guinea pig brain

scientific article published on 01 October 2005

Synthetic Disaccharide Standards Enable Quantitative Analysis of Stored Heparan Sulfate in MPS IIIA Murine Brain Regions

scientific article published on 18 July 2019

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

Treatment of canine fucosidosis by intracisternal enzyme infusion

scientific article published on 06 May 2011

Validation of a heparan sulfate-derived disaccharide as a marker of accumulation in murine mucopolysaccharidosis type IIIA.

scientific article

Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates

scientific article published on 22 October 2015

β-Mannosidosis in German Shepherd Dogs

scientific article published on 14 April 2019

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