Michael B. Tropak

Hospital for Sick Children — Canada

Medicine · Health Sciences

40h-index5.3kcitations99works0.52yr avg
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Research Topics

Lysosomal Storage Disorders Research(42), Glycosylation and Glycoproteins Research(30), Carbohydrate Chemistry and Synthesis(25), Cardiac Arrest and Resuscitation(12), Cardiac Ischemia and Reperfusion(12)

Publications99 total

A CNS-Directed, AAV9 Gene Therapy Restores Expression and Biochemical Function of Guanidinoacetate Methyltransferase in Models of GAMT Deficiency
International Journal of Molecular Sciences·2026Open Access
Validation and Optimization of a Stable Isotope-Labeled Substrate Assay for Measuring AGAT Activity
International Journal of Molecular Sciences·2024Open Access
Targeting AGAT gene expression – a drug screening approach for the treatment of GAMT deficiency
Expert Opinion on Drug Discovery·2024· 2 cited
Ribosome-stalling reporters suggest the N-terminus of AGAT acts as a creatine-dependent translation-arrest peptide
Molecular Genetics and Metabolism·2024
Evidence of an intracellular creatine-sensing mechanism that modulates creatine biosynthesis via AGAT expression in human HAP1 cells
Scientific Reports·2023· 8 citedOpen Access
Investigation of the creatine biosynthesis negative feedback loop via creatine mediated control of arginine:glycine amidinotransferase expression in human HAP1 cells implies an intracellular creatine sensing mechanism
Research Square (Research Square)·2023Open Access
Biallelic loss-of-function variants in RABGAP1 cause a novel neurodevelopmental syndrome
Genetics in Medicine·2022· 11 citedOpen Access
THE MECHANISM OF CREATINE MEDIATED DOWNREGULATION OF AGAT EXPRESSION
Molecular Genetics and Metabolism·2022· 2 cited
KINETIC ANALYSIS OF N-DEACETYLASE/N-SULFOTRANSFERASE (NDST1) INHIBITORS USING A FLUOROMETRIC COUPLED ENZYME ASSAY
Molecular Genetics and Metabolism·2022
Fluorometric coupled enzyme assay for N-sulfotransferase activity of N-deacetylase/N-sulfotransferase (NDST)
Glycobiology·2021· 9 cited
Novel Vector Design and Hexosaminidase Variant Enabling Self-Complementary Adeno-Associated Virus for the Treatment of Tay-Sachs Disease
UNC Libraries·2020Open Access
Novel Vector Design and Hexosaminidase Variant Enabling Self-Complementary Adeno-Associated Virus for the Treatment of Tay-Sachs Disease
Human Gene Therapy·2016· 43 citedOpen Access
Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo
Molecular Therapy — Methods & Clinical Development·2016· 50 citedOpen Access
Pyrimethamine Derivatives: Insight into Binding Mechanism and Improved Enhancement of Mutant β-<i>N</i>-acetylhexosaminidase Activity
Journal of Medicinal Chemistry·2015· 11 citedOpen Access
TMEM43 Mutation p.S358L Alters Intercalated Disc Protein Expression and Reduces Conduction Velocity in Arrhythmogenic Right Ventricular Cardiomyopathy
PLoS ONE·2014· 42 citedOpen Access
ChemInform Abstract: 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.
ChemInform·2014
Synthesis of 1,5-Dideoxy-1,5-iminoribitol <i>C</i>-Glycosides through a Nitrone–Olefin Cycloaddition Domino Strategy: Identification of Pharmacological Chaperones of Mutant Human Lysosomal β-Galactosidase
The Journal of Organic Chemistry·2014· 51 cited
Transcutaneous electrical nerve stimulation as a novel method of remote preconditioning: in vitro validation in an animal model and first human observations
Basic Research in Cardiology·2014· 50 cited
Remote Cardioprotection by Transfer of Coronary Effluent from Ischemic Preconditioned Rabbit Heart Preserves Mitochondrial Integrity and Function via Adenosine Receptor Activation
Cardiovascular Drugs and Therapy·2013· 52 cited
Interaction of δ and κ opioid receptors with adenosine A1 receptors mediates cardioprotection by remote ischemic preconditioning
Journal of Molecular and Cellular Cardiology·2013· 51 cited
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Frequent Co-authors

Don J. Mahuran(27), Andrew N. Redington(10), Stephen G. Withers(9), John Roder(8), Brigitte Rigat(7), Jing Li(6), John W. Callahan(6), Gustavo Maegawa(5), Joe T.R. Clarke(5), Justin Buttner(4), Cedric Manlhiot(4), R A Gravel(4), Sayuri Yonekawa(4), Georg Schitter(4), Andreas Steiner(4), Chris A. Tarling(4), Arnold Stütz(4), Tanja M. Wrodnigg(4), G. Kornhaber(3), Yoshitomo Hamuro(3)