Research Output 2008 2019

2019

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., Neumann, D., King, B., Beard, H., Trim, P., Winner, L., Bowen, A., Snel, M., Gomila, C., Ausseil, J., Mei, X., Giersch, L., Plavsic, M. & Laufer, R., 9 Dec 2019, In : Molecular Therapy - Methods and Clinical Development. 17, p. 174-187 14 p.

Research output: Contribution to journalArticle

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., Duplock, S., Snel, M., Hopwood, J. & Hemsley, K., 1 Dec 2019, In : Neuropathology and Applied Neurobiology. 45, 7, p. 715-731 17 p.

Research output: Contribution to journalArticle

Evaluation of Small Molecule Drug Uptake in Patient-Derived Prostate Cancer Explants by Mass Spectrometry

Mutuku, S. M., Trim, P., Prabhala, B. K., Irani, S., Bremert, K., Logan, J. M., Brooks, D. A., Stahl, J., Centenera, M. M., Snel, M. & Butler, L., 1 Dec 2019, In : Scientific Reports. 9, 1, 15008.

Research output: Contribution to journalArticle

1 Citation (Scopus)

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 journalArticle

2018
1 Citation (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., 20 Jun 2018, JIMD Reports. Springer, p. 91-101 11 p. (JIMD Reports; vol. 43).

Research output: Chapter in Book/Report/Conference proceedingChapter

Ion Mobility Separation Mass Spectrometry Imaging

Snel, M. F., 2018, (Accepted/In press) In : Comprehensive Analytical Chemistry.

Research output: Contribution to journalArticle

2 Citations (Scopus)

Lysosomal N-acetyltransferase interacts with ALIX and is detected in extracellular vesicles

Fedele, A. O., Isenmann, S., Kamei, M., Snel, M., Trim, P., Proud, C. & Hopwood, J., 1 Oct 2018, In : Biochimica et Biophysica Acta - Molecular Cell Research. 1865, 10, p. 1451-1464 14 p.

Research output: Contribution to journalArticle

3 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., 1 May 2018, In : Experimental Neurology. 303, p. 38-47 10 p.

Research output: Contribution to journalArticle

2017
3 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., 27 Apr 2017, (Accepted/In press) In : Journal of Inherited Metabolic Disease. 40, 5, p. 1-10 10 p.

Research output: Contribution to journalArticle

5 Citations (Scopus)

Inbred Mouse Populations Exhibit Intergenerational Changes in Intestinal Microbiota Composition and Function Following Introduction to a Facility

Choo, J. M., Trim, P. J., Leong, L. E. X., Abell, G. C. J., Brune, C., Jeffries, N., Wesselingh, S., Dear, T. N., Snel, M. F. & Rogers, G. B., 2017, In : Frontiers in Microbiology. 8, p. 608

Research output: Contribution to journalArticle

5 Citations (Scopus)

Rodent whole-body sectioning and MALDI mass spectrometry imaging

Trim, P. J., 2017, Methods in Molecular Biology. Humana Press Inc., Vol. 1618. p. 175-189 15 p. (Methods in Molecular Biology; vol. 1618).

Research output: Chapter in Book/Report/Conference proceedingChapter

4 Citations (Scopus)

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., 1 May 2017, In : Journal of Inherited Metabolic Disease. 40, 3, p. 443-453 11 p.

Research output: Contribution to journalArticle

2016
5 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., 1 Jul 2016, In : Neurobiology of Disease. 91, p. 143-154 12 p.

Research output: Contribution to journalArticle

6 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., 1 Apr 2016, In : Experimental Neurology. 278, p. 11-21 11 p.

Research output: Contribution to journalArticle

9 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., 1 May 2016, In : Journal of Neurochemistry. 137, 3, p. 409-422 14 p.

Research output: Contribution to journalArticle

29 Citations (Scopus)

Small molecule MALDI MS imaging: Current technologies and future challenges

Trim, P. J. & Snel, M. F., 15 Jul 2016, In : Methods. 104, p. 127-141 15 p.

Research output: Contribution to journalReview article

2015
16 Citations (Scopus)
21 Citations (Scopus)

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 journalArticle

12 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 journalArticle

Epithelial-to-Mesenchymal Transition as a Potential Target for Antineoplastic Therapies

Rao, SUSHMA. & Jayachandran, A., Nov 2015, In : Journal of Cancer Clinical Trials.

Research output: Contribution to journalEditorial

11 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., 1 Jan 2015, In : Journal of Inherited Metabolic Disease. 38, 2, p. 341-350 10 p.

Research output: Contribution to journalArticle

2014
18 Citations (Scopus)

A simple method for early age phenotype confirmation using toe tissue from a mouse model of MPS IIIA

Trim, P. J., Lau, A. A., Hopwood, J. J. & Snel, M. F., 30 Apr 2014, In : Rapid Communications in Mass Spectrometry. 28, 8, p. 933-938 6 p.

Research output: Contribution to journalArticle

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., 1 Jan 2014, In : European Journal of Neuroscience. 39, 12, p. 2139-2150 12 p.

Research output: Contribution to journalArticle

17 Citations (Scopus)

Liquid chromatography/electrospray ionisation-tandem mass spectrometry quantification of GM2 gangliosides in human peripheral cells and plasma

Fuller, M., Duplock, S., Hein, L. K., Rigat, B. A. & Mahuran, D. J., 1 Aug 2014, In : Analytical Biochemistry. 458, p. 20-26 7 p.

Research output: Contribution to journalArticle

2013
16 Citations (Scopus)

Selective reduction of bis(monoacylglycero)phosphate ameliorates the storage burden in a THP-1 macrophage model of Gaucher disease

Hein, L. K., Duplock, S. & Fuller, M., 1 Jun 2013, In : Journal of Lipid Research. 54, 6, p. 1691-1697 7 p.

Research output: Contribution to journalArticle

2012
18 Citations (Scopus)

Instrumentation and software for mass spectrometry imaging-Making the most of what you've got

Trim, P. J., Djidja, M. C., Muharib, T., Cole, L. M., Flinders, B., Carolan, V. A., Francese, S. & Clench, M. R., 30 Aug 2012, In : Journal of Proteomics. 75, 16, p. 4931-4940 10 p.

Research output: Contribution to journalArticle

2010
149 Citations (Scopus)

On-Tissue Protein Identification and Imaging by MALDI-Ion Mobility Mass Spectrometry

Stauber, J., MacAleese, L., Franck, J., Claude, E., Snel, M., Kaletas, B. K., Wiel, I. M. V. D., Wisztorski, M., Fournier, I. & Heeren, R. M. A., 1 Mar 2010, In : Journal of the American Society for Mass Spectrometry. 21, 3, p. 338-347 10 p.

Research output: Contribution to journalArticle

2009
97 Citations (Scopus)

MALDI-ion mobility separation-mass spectrometry imaging of glucose-regulated protein 78 kDa (Grp78) in human formalin-fixed, paraffin-embedded pancreatic adenocarcinoma tissue sections

Djidja, M. C., Claude, E., Snel, M. F., Scriven, P., Francese, S., Carolan, V. & Clench, M. R., 19 Oct 2009, In : Journal of Proteome Research. 8, 10, p. 4876-4884 9 p.

Research output: Contribution to journalArticle

2008
52 Citations (Scopus)

Effect of lysosomal storage on bis(monoacylglycero)phosphate

Meikle, P. J., Duplock, S., Blacklock, D., Whitfield, P. D., MacIntosh, G., Hopwood, J. J. & Fuller, M., 1 Apr 2008, In : Biochemical Journal. 411, 1, p. 71-78 8 p.

Research output: Contribution to journalArticle

40 Citations (Scopus)

Lipid composition of microdomains is altered in a cell model of Gaucher disease

Hein, L. K., Duplock, S., Hopwood, J. J. & Fuller, M., 1 Aug 2008, In : Journal of Lipid Research. 49, 8, p. 1725-1734 10 p.

Research output: Contribution to journalArticle