Research output per year
Research output per year
- 1 - 50 out of 85 results
Search results
-
2024
Application of diceCT to Study the Development of the Zika Virus-Infected Mouse Brain
Green, A. L., Cowell, E. C., Carr, L. M., Hemsley, K., Sherratt, E., Collins-Praino, L. E. & Carr, J. M., Aug 2024, In: Viruses. 16, 8, 19 p., 1330.Research output: Contribution to journal › Article › peer-review
Open AccessFile9 Downloads (Pure) -
Compromised transcription-mRNA export factor THOC2 causes R-loop accumulation, DNA damage and adverse neurodevelopment
Bhattacharjee, R., Jolly, L. A., Corbett, M. A., Wee, I. C., Rao, S. R., Gardner, A. E., Ritchie, T., van Hugte, E. J. H., Ciptasari, U., Piltz, S., Noll, J. E., Nazri, N., van Eyk, C. L., White, M., Fornarino, D., Poulton, C., Baynam, G., Collins-Praino, L. E., Snel, M. F., Nadif Kasri, N., & 4 others, 8 Feb 2024, In: Nature Communications. 15, 1, 25 p., 1210.Research output: Contribution to journal › Article › peer-review
Open AccessFile39 Downloads (Pure) -
Drosophila melanogaster models of MPS IIIC (Hgsnat-deficiency) highlight the role of glia in disease presentation
Hewson, L., Choo, A., Webber, D. L., Trim, P. J., Snel, M. F., Fedele, A. O., Hopwood, J. J., Hemsley, K. M. & O'Keefe, L. V., Mar 2024, In: Journal of Inherited Metabolic Disease. 47, 2, p. 340-354 15 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile26 Downloads (Pure) -
Evaluation of neuroretina following i.v. or intra-CSF AAV9 gene replacement in mice with MPS IIIA, a childhood dementia
Beard, H., Winner, L., Shoubridge, A., Parkinson-Lawrence, E., Lau, A. A., Mubarokah, S. N., Lance, T-R., King, B., Scott, W., Snel, M. F., Trim, P. J. & Hemsley, K. M., Aug 2024, In: CNS Neuroscience and Therapeutics. 30, 8, 13 p., e14919.Research output: Contribution to journal › Article › peer-review
Open AccessFile7 Downloads (Pure) -
Filipin complex-reactive brain lesions: A cautionary tale
Lau, A. A., Trim, P. J., King, B. M., Hassiotis, S., Hung, Y. H., Bush, A. I., Snel, M. F. & Hemsley, K. M., Feb 2024, In: Neuropathology and Applied Neurobiology. 50, 1, 8 p., e12950.Research output: Contribution to journal › Article › peer-review
-
Photobiomodulation in the infrared spectrum reverses the expansion of circulating natural killer cells and brain microglial activation in Sanfilippo mice
Lau, A. A., Jin, K., Beard, H., Windram, T., Xie, K., O’Brien, J. A., Neumann, D., King, B. M., Snel, M. F., Trim, P. J., Mitrofanis, J., Hemsley, K. M. & Austin, P. J., 7 Jun 2024, (E-pub ahead of print) In: Journal of Neurochemistry. p. 1-23 23 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile21 Downloads (Pure) -
Repetitive, non-invasive imaging of neurodegeneration, and prevention of it with gene replacement, in mice with Sanfilippo syndrome.
Hemsley, K. M., Beard, H., Chidlow, G., Mammone, T., Winner, L. K., Neumann, D., King, B., Snel, M. F., Trim, P. J. & Casson, R. J., Jan 2024, In: Experimental Neurology. 371, 12 p., 114610.Research output: Contribution to journal › Article › peer-review
1 Citation (Scopus) -
Striking and widespread microglial activation in the brains of Southdown lambs with type II glucocerebrosidosis (neuronopathic Gaucher disease)
Finnie, J., Hemsley, K., Manavis, J., Beard, H., Brealey, J., Robertson, T. & Blumbergs, P., Nov 2024, In: Journal of Comparative Pathology. 215, p. 10-13 4 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile1 Downloads (Pure) -
2023
A lower motor neuron disease in takahē (Porphyrio hochstetteri) is an endoplasmic reticulum storage disease
Jolly, RD., Perrott, MR., Alley, MR., Hunter, SA., Pas, A., Beard, H., Hemsley, KM. & Greaves, G., 2023, In: New Zealand Veterinary Journal. 71, 4, p. 186-193 8 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile39 Downloads (Pure) -
Biomarkers for predicting disease course in Sanfilippo syndrome: An urgent unmet need in childhood-onset dementia
Winner, L. K., Rogers, M-L., Snel, M. F. & Hemsley, K. M., Aug 2023, In: Journal of Neurochemistry. 166, 3, p. 481-496 16 p.Research output: Contribution to journal › Review article › peer-review
Open AccessFile2 Citations (Scopus)107 Downloads (Pure) -
Substrate reduction therapy in a Drosophila melanogaster model of Sanfilippo syndrome
Tan, S. L., Hewson, L. J., Mustaffar, N. F., He, Q. Q., Wimmer, N., Trim, P. J., King, B., Snel, M. F., Hemsley, K. M., Ferro, V., O'Keefe, L. V. & Lau, A. A., 28 Mar 2023, bioRxiv, Cold Spring Harbor Laboratory, 27 p.Research output: Working paper/Preprint › Preprint
-
The ovine Type II Gaucher disease model recapitulates aspects of human brain disease
Winner, L. K., Beard, H., Karageorgos, L., Smith, N. J., Hopwood, J. J. & Hemsley, K. M., Apr 2023, In: Biochimica et Biophysica Acta. Molecular Basis of Disease. 1869, 4, 11 p., 166658.Research output: Contribution to journal › Article › peer-review
2 Citations (Scopus) -
2022
Lysosomal gene Hexb displays haploinsufficiency in a knock-in mouse model of Alzheimer's disease
Whyte, L. S., Fourrier, C., Hassiotis, S., Lau, A. A., Trim, P. J., Hein, L. K., Hattersley, K. J., Bensalem, J., Hopwood, J. J., Hemsley, K. M. & Sargeant, T. J., Jun 2022, In: IBRO Neuroscience Reports. 12, p. 131-141 11 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile8 Citations (Scopus)50 Downloads (Pure) -
2021
Animal medical genetics: a historical perspective on more than 50 years of research into genetic disorders of animals at Massey University
Jolly, R. D., Dittmer, K. E., Jones, B. R., Worth, A. J., Thompson, K. G., Johnstone, A. C., Palmer, D. N., Van de Water, N. S., Hemsley, K. M., Garrick, D. J., Winchester, B. G. & Walkley, S. U., 2021, In: New Zealand Veterinary Journal. 69, 5, p. 255-266 12 p.Research output: Contribution to journal › Review article › peer-review
-
Is SGSH heterozygosity a risk factor for early-onset neurodegenerative disease?
Douglass, M. L., Beard, H., Shoubridge, A., Nazri, N., King, B., Trim, P. J., Duplock, S. K., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., May 2021, In: Journal of Inherited Metabolic Disease. 44, 3, p. 763-776 14 p.Research output: Contribution to journal › Article › peer-review
3 Citations (Scopus) -
Mucopolysaccharidosis II (MPS II) in a free-living kaka (nestor meridionalis) in New Zealand
Jolly, R. D., Hunter, S. A., Alley, M. R., King, B. M., Lau, A. A., Trim, P. J., Snel, M. F. & Hemsley, K. M., Oct 2021, In: Journal of Wildlife Diseases. 57, 4, p. 884-890 7 p.Research output: Contribution to journal › Article › peer-review
3 Citations (Scopus) -
2020
AAVrh10 Vector Corrects Disease Pathology in MPS IIIA Mice and Achieves Widespread Distribution of SGSH in Large Animal Brains
Hocquemiller, M., Hemsley, K. M., Douglass, M. L., Tamang, S. J., Neumann, D., King, B. M., Beard, H., Trim, P. J., Winner, L. K., Lau, A. A., Snel, M. F., Gomila, C., Ausseil, J., Mei, X., Giersch, L., Plavsic, M. & Laufer, R., 12 Jun 2020, In: Molecular Therapy: Methods and Clinical Development. 17, p. 174-187 14 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile25 Citations (Scopus)101 Downloads (Pure) -
Is the eye a window to the brain in Sanfilippo syndrome?
Beard, H., Chidlow, G., Neumann, D., Nazri, N., Douglass, M., Trim, P. J., Snel, M. F., Casson, R. J. & Hemsley, K. M., Dec 2020, In: Acta Neuropathologica Communications. 8, 16 p., 194.Research output: Contribution to journal › Article › peer-review
Open AccessFile6 Citations (Scopus)36 Downloads (Pure) -
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
O'Connell, A. B., Kuchel, T. R., Perumal, S. R., Sherwood, V., Neumann, D., Finnie, J. W., Hemsley, K. M. & Morton, A. J., 1 Oct 2020, In: Journal of neuropathology and experimental neurology. 79, 10, p. 1084-1092 9 p.Research output: Contribution to journal › Article › peer-review
4 Citations (Scopus) -
Lysosomal Dysregulation in the Murine AppNL-G-F/NL-G-F Model of Alzheimer's Disease
Whyte, L. S., Hassiotis, S., Hattersley, K. J., Hemsley, K. M., Hopwood, J. J., Lau, A. A. & Sargeant, T. J., 1 Mar 2020, In: Neuroscience. 429, p. 143-155 13 p.Research output: Contribution to journal › Article › peer-review
12 Citations (Scopus) -
2019
Canine adenoviral vector-mediated gene transfer to the guinea pig brain
Lau, A. A., Hopwood, J. J. & Hemsley, K. M., Sept 2019, In: Gene Reports. 16, 5 p., 100432.Research output: Contribution to journal › Article › peer-review
1 Citation (Scopus) -
Early disease course is unaltered in mucopolysaccharidosis type IIIA (MPS IIIA) mice lacking α-synuclein
Soe, K., Beard, H., Neumann, D., Trim, P. J., Duplock, S., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., 1 Dec 2019, In: Neuropathology and Applied Neurobiology. 45, 7, p. 715-731 17 p.Research output: Contribution to journal › Article › peer-review
7 Citations (Scopus) -
Evaluation of disease lesions in the developing canine MPS IIIA brain
Winner, L. K., Marshall, N. R., Jolly, R. D., Trim, P. J., Duplock, S. K., Snel, M. F. & Hemsley, K. M., 15 Jan 2019, In: JIMD Reports. 43, p. 91-101 11 p.Research output: Contribution to journal › Article › peer-review
9 Citations (Scopus) -
Neurological Examination of Sheep (Ovis aries) with Unilateral and Bilateral Quinolinic Acid Lesions of the Striatum Assessed by Magnetic Resonance Imaging
O'Connell, A., Sinnott, B., Kuchel, T. R., Perumal, S. R., Fraser, C. K., Hemsley, K. M. & Morton, A. J., 6 Aug 2019, In: Journal of Neurology and Experimental Neuroscience. 5, 2, p. 56-67 12 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile71 Downloads (Pure) -
Synthetic Disaccharide Standards Enable Quantitative Analysis of Stored Heparan Sulfate in MPS IIIA Murine Brain Regions
He, Q. Q., Trim, P. J., Lau, A. A., King, B. M., Hopwood, J. J., Hemsley, K. M., Snel, M. F. & Ferro, V., 21 Aug 2019, In: ACS Chemical Neuroscience. 10, 8, p. 3847-3858 12 p.Research output: Contribution to journal › Article › peer-review
13 Citations (Scopus) -
β-Mannosidosis in German Shepherd Dogs
Jolly, R. D., Dittmer, K. E., Garrick, D. J., Chernyavtseva, A., Hemsley, K. M., King, B., Fietz, M., Shackleton, N. M., Fairley, R. & Wylie, K., Sept 2019, In: Veterinary Pathology. 56, 5, p. 743-748 6 p.Research output: Contribution to journal › Article › peer-review
8 Citations (Scopus) -
2018
MPS-IIIA mice acquire autistic behaviours with age
Lau, A. A., Tamang, S. J. & Hemsley, K. M., Jul 2018, In: Journal of Inherited Metabolic Disease. 41, 4, p. 669-677 9 p.Research output: Contribution to journal › Article › peer-review
11 Citations (Scopus) -
Neuronal-specific impairment of heparan sulfate degradation in Drosophila reveals pathogenic mechanisms for Mucopolysaccharidosis type IIIA
Webber, D. L., Choo, A., Hewson, L. J., Trim, P. J., Snel, M. F., Hopwood, J. J., Richards, R. I., Hemsley, K. M. & O'Keefe, L. V., May 2018, In: Experimental Neurology. 303, p. 38-47 10 p.Research output: Contribution to journal › Article › peer-review
16 Citations (Scopus) -
Reduction in open field activity in the absence of memory deficits in the AppNL−G−F knock-in mouse model of Alzheimer's disease
Whyte, L. S., Hemsley, K. M., Lau, A. A., Hassiotis, S., Saito, T., Saido, T. C., Hopwood, J. J. & Sargeant, T. J., 15 Jan 2018, In: Behavioural Brain Research. 336, p. 177-181 5 p.Research output: Contribution to journal › Article › peer-review
50 Citations (Scopus) -
SAHMRI Consumer Register Project: Consultation Summary, Recommendations & Functional Design
Bevan, A., Janke, A., Miller, C., Yeung, D. T., Kay, D., Kerrins, E., Croucher, H., Thomas, N., Butters, J., Mickan, K., Hanna, K., Hemsley, K., Helibron, L., Coleman, M., Cobb, E., Musker, M., Middleton, P., Pinero de Plaza, M. A. & Wesselingh, S., Feb 2018, Adelaide, S. A. : South Australian Health and Medical Research Institute. 38 p.Research output: Book/Report › Commissioned report › peer-review
-
2017
Adeno-associated viral gene therapy for mucopolysaccharidoses exhibiting neurodegeneration
Lau, A. A. & Hemsley, K. M., Oct 2017, In: Journal of Molecular Medicine. 95, 10, p. 1043-1052 10 p.Research output: Contribution to journal › Review article › peer-review
7 Citations (Scopus) -
A novel conditional Sgsh knockout mouse model recapitulates phenotypic and neuropathic deficits of Sanfilippo syndrome
Lau, A. A., King, B. M., Thorsen, C. L., Hassiotis, S., Beard, H., Trim, P. J., Whyte, L. S., Tamang, S. J., Duplock, S. K., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., Sept 2017, In: Journal of Inherited Metabolic Disease. 40, 5, p. 715-724 10 p.Research output: Contribution to journal › Article › peer-review
12 Citations (Scopus) -
Axonal dystrophy in the brain of mice with Sanfilippo syndrome
Beard, H., Hassiotis, S., Gai, W. P., Parkinson-Lawrence, E., Hopwood, J. J. & Hemsley, K. M., Sept 2017, In: Experimental Neurology. 295, p. 243-255 13 p.Research output: Contribution to journal › Article › peer-review
32 Citations (Scopus) -
Endo-lysosomal and autophagic dysfunction: a driving factor in Alzheimer's disease?
Whyte, L. S., Lau, A. A., Hemsley, K. M., Hopwood, J. J. & Sargeant, T. J., 1 Mar 2017, In: Journal of Neurochemistry. 140, 5, p. 703-717 15 p.Research output: Contribution to journal › Review article › peer-review
105 Citations (Scopus) -
Intracerebral gene therapy for mucopolysaccharidosis type IIIB syndrome
Hemsley, K. & Lau, A., Sept 2017, In: The Lancet Neurology. 16, 9, p. 681-682 2 p.Research output: Contribution to journal › Comment/debate
-
Slow, continuous enzyme replacement via spinal CSF in dogs with the paediatric-onset neurodegenerative disease, MPS IIIA
King, B., Marshall, N. R., Hassiotis, S., Trim, P. J., Tucker, J., Hattersley, K., Snel, M. F., Jolly, R. D., Hopwood, J. J. & Hemsley, K. M., May 2017, In: Journal of Inherited Metabolic Disease. 40, 3, p. 443-453 11 p.Research output: Contribution to journal › Article › peer-review
16 Citations (Scopus) -
2016
A preclinical study evaluating AAVrh10-based gene therapy for sanfilippo syndrome
Winner, L. K., Beard, H., Hassiotis, S., Lau, A. A., Luck, A. J., Hopwood, J. J. & Hemsley, K. M., 1 May 2016, In: Human Gene Therapy . 27, 5, p. 363-375 13 p.Research output: Contribution to journal › Article › peer-review
40 Citations (Scopus) -
Continual low-dose infusion of sulfamidase is superior to intermittent high-dose delivery in ameliorating neuropathology in the MPS IIIA mouse brain
Beard, H., Hassiotis, S., Luck, A. J., Rozaklis, T., Hopwood, J. J. & Hemsley, K. M., 8 Sept 2016, In: JIMD Reports. 29, p. 59-68 10 p.Research output: Contribution to journal › Article › peer-review
6 Citations (Scopus) -
Glycosphingolipid analysis in a naturally occurring ovine model of acute neuronopathic Gaucher disease
Karageorgos, L., Hein, L., Rozaklis, T., Adams, M., Duplock, S., Snel, M., Hemsley, K., Kuchel, T., Smith, N. & Hopwood, J. J., Jul 2016, In: Neurobiology of Disease. 91, p. 143-154 12 p.Research output: Contribution to journal › Article › peer-review
15 Citations (Scopus) -
Low-dose, continual enzyme delivery ameliorates some aspects of established brain disease in a mouse model of a childhood-onset neurodegenerative disorder
King, B., Setford, M. L., Hassiotis, S., Trim, P. J., Duplock, S., Tucker, J. N., Hattersley, K., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., Apr 2016, In: Experimental Neurology. 278, p. 11-21 11 p.Research output: Contribution to journal › Article › peer-review
11 Citations (Scopus) -
Low-dose, continuous enzyme replacement therapy ameliorates brain pathology in the neurodegenerative lysosomal disorder mucopolysaccharidosis type IIIA
King, B., Hassiotis, S., Rozaklis, T., Beard, H., Trim, P. J., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., May 2016, In: Journal of Neurochemistry. 137, 3, p. 409-422 14 p.Research output: Contribution to journal › Article › peer-review
14 Citations (Scopus) -
2015
Delivery of therapeutic protein for prevention of neurodegenerative changes: Comparison of different CSF-delivery methods
Marshall, N. R., Hassiotis, S., King, B., Rozaklis, T., Trim, P. J., Duplock, S. K., Winner, L. K., Beard, H., Snel, M. F., Jolly, R. D., Hopwood, J. J. & Hemsley, K. M., 1 Jan 2015, In: Experimental Neurology. 263, p. 79-90 12 p.Research output: Contribution to journal › Article › peer-review
26 Citations (Scopus) -
Determination of the role of injection site on the efficacy of intra-CSF enzyme replacement therapy in MPS IIIA mice
Beard, H., Luck, A. J., Hassiotis, S., King, B., Trim, P. J., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., 1 Jan 2015, In: Molecular Genetics and Metabolism. 115, 1, p. 33-40 8 p.Research output: Contribution to journal › Article › peer-review
23 Citations (Scopus) -
Evaluation of enzyme dose and dose-frequency in ameliorating substrate accumulation in MPS IIIA Huntaway dog brain
King, B., Marshall, N., Beard, H., Hassiotis, S., Trim, P. J., Snel, M. F., Rozaklis, T., Jolly, R. D., Hopwood, J. J. & Hemsley, K. M., Mar 2015, In: Journal of Inherited Metabolic Disease. 38, 2, p. 341-350 10 p.Research output: Contribution to journal › Article › peer-review
18 Citations (Scopus) -
Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates
Whyte, L. S., Hopwood, J. J., Hemsley, K. M. & Lau, A. A., 1 Dec 2015, In: Molecular Genetics and Metabolism Reports. 5, p. 60-62 3 p.Research output: Contribution to journal › Article › peer-review
Open AccessFile6 Citations (Scopus)27 Downloads (Pure) -
2014
Development of cerebellar pathology in the canine model of mucopolysaccharidosis type IIIA (MPS IIIA)
Hassiotis, S., Jolly, R. D. & Hemsley, K. M., 1 Dec 2014, In: Molecular Genetics and Metabolism. 113, 4, p. 283-293 11 p.Research output: Contribution to journal › Article › peer-review
9 Citations (Scopus) -
Disease stage determines the efficacy of treatment of a paediatric neurodegenerative disease
Hassiotis, S., Beard, H., Luck, A., Trim, P. J., King, B., Snel, M. F., Hopwood, J. J. & Hemsley, K. M., Jun 2014, In: European Journal of Neuroscience. 39, 12, p. 2139-2150 12 p.Research output: Contribution to journal › Article › peer-review
17 Citations (Scopus) -
2013
Intracisternal enzyme replacement therapy in lysosomal storage diseases: Dispersal pathways, regional enzyme concentrations and the effect of posttreatment posture
Jolly, R. D., Marshall, N. R., Marshall, J., Hartman, A., Hemsley, K. M. & Winner, L. K., Oct 2013, In: Neuropathology and Applied Neurobiology. 39, 6, p. 681-692 12 p.Research output: Contribution to journal › Article › peer-review
11 Citations (Scopus) -
Neonatal bone marrow transplantation in MPS IIIA Mice
Lau, A. A., Shamsani, N. J., Winner, L. K., Hassiotis, S., King, B. M., Hopwood, J. J. & Hemsley, K. M., 1 Jan 2013, JIMD Reports: Case and Research Reports, 2012/5. Zschocke, J., Gibson, K., Brown, G., Morava, E. & Peters, V. (eds.). Berlin: Springer , Vol. 8. p. 121-132 12 p. (JIMD Reports; vol. 8).Research output: Chapter in Book/Report/Conference proceeding › Chapter › peer-review
Open Access21 Citations (Scopus) -
2012
Abnormal gangliosides are localized in lipid rafts in Sanfilippo (MPS3a) mouse brain
Dawson, G., Fuller, M., Helmsley, K. M. & Hopwood, J. J., 1 Jun 2012, In: Neurochemical Research. 37, 6, p. 1372-1380 9 p.Research output: Contribution to journal › Article › peer-review
24 Citations (Scopus)