PMC Articles for MedGen (Select 1095561) - PMC

Select item 102985241.

Glucosylceramide Synthase Inhibitors Induce Ceramide Accumulation and Sensitize H3K27 Mutant Diffuse Midline Glioma to Irradiation

Khalifa El Malki, Pia Wehling, Francesca Alt, Roger Sandhoff, Sebastian Zahnreich, Arsenij Ustjanzew, Carolin Wilzius, Marc A. Brockmann, Arthur Wingerter, Alexandra Russo, Olaf Beck, Clemens Sommer, Malte Ottenhausen, Katrin B. M. Frauenknecht, Claudia Paret, Jörg Faber

Int J Mol Sci. 2023 Jun; 24(12): 9905. Published online 2023 Jun 8. doi: 10.3390/ijms24129905

PMCID:: PMC10298524

Abstract Article PDF–5.1M

Select item 100699642.

Functional TFEB activation characterizes multiple models of renal cystic disease and loss of polycystin-1

Jonathan M. Shillingford, James A. Shayman

Am J Physiol Renal Physiol. 2023 Apr 1; 324(4): F404–F422. Published online 2023 Feb 16. doi: 10.1152/ajprenal.00237.2022

PMCID:: PMC10069964

Abstract Article

Select item 85448343.

Cellular and biochemical response to chaperone versus substrate reduction therapies in neuropathic Gaucher disease

Margarita M. Ivanova, Julia Dao, Neil Kasaci, Benjamin Adewale, Shaista Nazari, Lauren Noll, Jacqueline Fikry, Armaghan Hafez Sanati, Ozlem Goker-Alpan

PLoS One. 2021; 16(10): e0247211. Published online 2021 Oct 25. doi: 10.1371/journal.pone.0247211

PMCID:: PMC8544834

Abstract Article PDF–3.3M

Select item 79044754.

Glucosylceramide synthase inhibitors prevent replication of SARS-CoV-2 and influenza virus

Einat B. Vitner, Hagit Achdout, Roy Avraham, Boaz Politi, Lilach Cherry, Hadas Tamir, Yfat Yahalom-Ronen, Nir Paran, Sharon Melamed, Noam Erez, Tomer Israely

J Biol Chem. 2021 Jan-Jun; 296: 100470. Published online 2021 Feb 25. doi: 10.1016/j.jbc.2021.100470

PMCID:: PMC7904475

Abstract Article PDF–1017K

Select item 32691555.

Property-based design of a glucosylceramide synthase inhibitor that reduces glucosylceramide in the brain

Scott D. Larsen, Michael W. Wilson, Akira Abe, Liming Shu, Christopher H. George, Paul Kirchhoff, H. D. Hollis Showalter, Jianming Xiang, Richard F. Keep, James A. Shayman

J Lipid Res. 2012 Feb; 53(2): 282–291. doi: 10.1194/jlr.M021261

PMCID:: PMC3269155

Abstract Article PDF–753K

Select item 29913506.

Substrate Reduction Augments the Efficacy of Enzyme Therapy in a Mouse Model of Fabry Disease

John Marshall, Karen M. Ashe, Dinesh Bangari, KerryAnne McEachern, Wei-Lien Chuang, Joshua Pacheco, Diane P. Copeland, Robert J. Desnick, James A. Shayman, Ronald K. Scheule, Seng H. Cheng

PLoS One. 2010; 5(11): e15033. Published online 2010 Nov 24. doi: 10.1371/journal.pone.0015033

PMCID:: PMC2991350

Abstract Article PDF–1.5M

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