Výsledky vyhledávání - "Don J. Mahuran"

Akademický článek

NDST1 Preferred Promoter Confirmation and Identification of Corresponding Transcriptional Inhibitors as Substrate Reduction Agents for Multiple Mucopolysaccharidosis Disorders.

Autor: Ilona Tkachyova, Xiaolian Fan, Anne-Marie LamHonWah, Bohdana Fedyshyn, Ingrid Tein, Don J Mahuran, Andreas Schulze

Publikováno v: PLoS ONE, Vol 11, Iss 9, p e0162145 (2016)

The stepwise degradation of glycosaminoglycans (GAGs) is accomplished by twelve lysosomal enzymes. Deficiency in any of these enzymes will result in the accumulation of the intermediate substrates on the pathway to the complete turnover of GAGs. The

Externí odkaz: https://doaj.org/article/be8e53cea7c14829b03de5054dfd5065

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Akademický článek

In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 ganglioside.

Autor: Incilay Sinici, Sayuri Yonekawa, Ilona Tkachyova, Steven J Gray, R Jude Samulski, Warren Wakarchuk, Brian L Mark, Don J Mahuran

Publikováno v: PLoS ONE, Vol 8, Iss 3, p e57908 (2013)

The hydrolysis in lysosomes of GM2 ganglioside to GM3 ganglioside requires the correct synthesis, intracellular assembly and transport of three separate gene products; i.e., the alpha and beta subunits of heterodimeric beta-hexosaminidase A, E.C. # 3

Externí odkaz: https://doaj.org/article/4df25f80c5ab47469e0a8236857830c9

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Akademický článek

Impaired glucose tolerance in a mouse model of sidt2 deficiency.

Autor: Jialin Gao, Xuefan Gu, Don J Mahuran, Zhugang Wang, Huiwen Zhang

Publikováno v: PLoS ONE, Vol 8, Iss 6, p e66139 (2013)

Sidt2 was identified as a novel integral lysosomal membrane protein recently. We generated global Sidt2 knockout mice by gene targeting. These mice have a comparatively higher random and fasting glucose concentration. Intraperitoneal and oral glucose

Externí odkaz: https://doaj.org/article/4930339b9c4942e49bce05b9a7512b57

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Ambroxol as a novel disease-modifying treatment for Parkinson’s disease dementia: protocol for a single-centre, randomized, double-blind, placebo-controlled trial

Autor: Michael Borrie, C. R.A. Silveira, Robert A. Hegele, Jennie Wells, J. MacKinley, Penny A. MacDonald, Mandar Jog, Mary E. Jenkins, Don J. Mahuran, Stephen H. Pasternak, C. A. Rupar, Sarah A. Morrow, Kristy Coleman, Robert Bartha, Elizabeth Finger, R. G. Tirona, Guangyong Zou, Z. Li

Publikováno v: BMC Neurology, Vol 19, Iss 1, Pp 1-10 (2019)
Medical Biophysics Publications
BMC Neurology

© 2019 The Author(s). Background: Currently there are no disease-modifying treatments for Parkinson's disease dementia (PDD), a condition linked to aggregation of the protein α-synuclein in subcortical and cortical brain areas. One of the leading g

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f846bf4a3d9f36571dbad625b1f012bd
https://doi.org/10.1186/s12883-019-1252-3

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Akademický článek

Synthesis of lipophilic 1-deoxygalactonojirimycin derivatives as D-galactosidase inhibitors

Autor: Georg Schitter, Elisabeth Scheucher, Andreas J. Steiner, Arnold E. Stütz, Martin Thonhofer, Chris A. Tarling, Stephen G. Withers, Jacqueline Wicki, Katrin Fantur, Eduard Paschke, Don J. Mahuran, Brigitte A. Rigat, Michael Tropak, Tanja M. Wrodnigg

Publikováno v: Beilstein Journal of Organic Chemistry, Vol 6, Iss 1, p 21 (2010)

N-Alkylation at the ring nitrogen of the D-galactosidase inhibitor 1-deoxygalactonojirimycin with a functionalised C6 alkyl chain followed by modification with different aromatic substituents provided lipophilic 1-deoxygalactonojirimycin derivatives

Externí odkaz: https://doaj.org/article/758f56058e784db1bcd1da10f98431f3

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Pyrimethamine Derivatives: Insight into Binding Mechanism and Improved Enhancement of Mutant β-N-acetylhexosaminidase Activity

Autor: Brigitte Rigat, Matthew R. Smith, Hee-Jong Hwang, Virender S. Aulakh, Don J. Mahuran, Sayuri Yonekawa, Jianmin Zhang, Marco A. Ciufolini, Michael B. Tropak

Publikováno v: Journal of Medicinal Chemistry. 58:4483-4493

In order to identify structural features of pyrimethamine (5-(4-chlorophenyl)-6-ethylpyrimidine-2,4-diamine) that contribute to its inhibitory activity (IC50 value) and chaperoning efficacy toward β-N-acetylhexosaminidase, derivatives of the compoun

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4d7e3126c7608f824e21f6110970c582
https://doi.org/10.1021/jm5017895

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

Autor: Brigitte Rigat, Don J. Mahuran, Maria Fuller, Stephen Duplock, Leanne K. Hein

Publikováno v: Analytical Biochemistry. 458:20-26

GM2 gangliosidosis is a group of inherited neurodegenerative disorders resulting primarily from the excessive accumulation of GM2 gangliosides (GM2) in neuronal cells. As biomarkers for categorising patients and monitoring the effectiveness of develo

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::787afa0c5d76626bf345be3a89d5bf24
https://doi.org/10.1016/j.ab.2014.04.018

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Synthesis of 1,5-Dideoxy-1,5-iminoribitol C-Glycosides through a Nitrone–Olefin Cycloaddition Domino Strategy: Identification of Pharmacological Chaperones of Mutant Human Lysosomal β-Galactosidase

Autor: Michael B. Tropak, Balu A. Chopade, Sayuri Yonekawa, Dhiraj P. Sonawane, Pramod R. Markad, Omprakash Bande, Don J. Mahuran, Dilip D. Dhavale, Aloysius Siriwardena, Sougata Ghosh

Publikováno v: The Journal of Organic Chemistry. 79:4398-4404

We report herein a newly developed domino reaction that facilitates the synthesis of new 1,5-dideoxy-1,5-iminoribitol iminosugar C-glycosides 7a-e and 8. The key intermediate in this approach is a six-membered cyclic sugar nitrone that is generated i

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9753ca5bffa6d10c3b5d7fce8c939309
https://doi.org/10.1021/jo500328u

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Novel Vector Design and Hexosaminidase Variant Enabling Self-Complementary Adeno-Associated Virus for the Treatment of Tay-Sachs Disease

Autor: Patrick Thompson, Michael David Kaytor, Michael B. Tropak, Jagdeep S. Walia, John G. Keimel, Sahana Nagabhushan Kalburgi, Subha Karumuthil-Melethil, Brian L. Mark, Don J. Mahuran, Steven J. Gray

GM2 gangliosidosis is a family of three genetic neurodegenerative disorders caused by the accumulation of GM2 ganglioside (GM2) in neuronal tissue. Two of these are due to the deficiency of the heterodimeric (α–β), “A” isoenzyme of lysosomal

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::36beefde55a72fc28286189393911820
https://europepmc.org/articles/PMC5349231/

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Evaluation of N-nonyl-deoxygalactonojirimycin as a pharmacological chaperone for human GM1 gangliosidosis leads to identification of a feline model suitable for testing enzyme enhancement therapy

Autor: Michael B. Tropak, Douglas R. Martin, John W. Callahan, Daphne Benedict, Ellen Crushell, Don J. Mahuran, Brigitte Rigat, Justin D. Buttner

Publikováno v: Molecular Genetics and Metabolism. 107:203-212

Deficiencies of lysosomal β-D-galactosidase can result in GM1 gangliosidosis, a severe neurodegenerative disease characterized by massive neuronal storage of GM1 ganglioside in the brain. Currently there are no available therapies that can even slow

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e277ba31a370c0b1fcb1969466e1f803
https://doi.org/10.1016/j.ymgme.2012.06.007

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