Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease von Marshall, John, Ashe, Karen M, Bangari, Dinesh, McEachern, KerryAnne, Chuang, Wei-Lien, Pacheco, Joshua, Copeland, Diane P, Desnick, Robert J, Shayman, James A, Scheule, Ronald K, Cheng, Seng H

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Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease von Ashe, Karen M, Bangari, Dinesh, Li, Lingyun, Cabrera-Salazar, Mario A, Bercury, Scott D, Nietupski, Jennifer B, Cooper, Christopher G F, Aerts, Johannes M F G, Lee, Edward R, Copeland, Diane P, Cheng, Seng H, Scheule, Ronald K, Marshall, John

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A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease von McEachern, Kerry Anne, Fung, John, Komarnitsky, Svetlana, Siegel, Craig S., Chuang, Wei-Lien, Hutto, Elizabeth, Shayman, James A., Grabowski, Gregory A., Aerts, Johannes M.F.G., Cheng, Seng H., Copeland, Diane P., Marshall, John

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Systemic delivery of a glucosylceramide synthase inhibitor reduces CNS substrates and increases lifespan in a mouse model of type 2 Gaucher disease von Cabrera-Salazar, Mario A, Deriso, Matthew, Bercury, Scott D, Li, Lingyun, Lydon, John T, Weber, William, Pande, Nilesh, Cromwell, Mandy A, Copeland, Diane, Leonard, John, Cheng, Seng H, Scheule, Ronald K

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Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann–Pick C mice von Nietupski, Jennifer B., Pacheco, Joshua J., Chuang, Wei-Lien, Maratea, Kimberly, Li, Lingyun, Foley, Joseph, Ashe, Karen M., Cooper, Christopher G.F., Aerts, Johannes M.F.G., Copeland, Diane P., Scheule, Ronald K., Cheng, Seng H., Marshall, John

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Dual-Action Lipophilic Iminosugar Improves Glycemic Control in Obese Rodents by Reduction of Visceral Glycosphingolipids and Buffering of Carbohydrate Assimilation von Wennekes, Tom, Meijer, Alfred J, Groen, Albert K, Boot, Rolf G, Groener, Johanna E, van Eijk, Marco, Ottenhoff, Roelof, Bijl, Nora, Ghauharali, Karen, Song, Hang, O’Shea, Tom J, Liu, Hanlan, Yew, Nelson, Copeland, Diane, van den Berg, Richard J, van der Marel, Gijsbert A, Overkleeft, Herman S, Aerts, Johannes M

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A Glucosylceramide Synthase Inhibitor Protects Rats Against the Cytotoxic Effects of Shiga Toxin 2 von Silberstein, Claudia, Lucero, María S, Zotta, Elsa, Copeland, Diane P, Lingyun, Li, Repetto, Horacio A, Ibarra, Cristina

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Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy von Marshall, John, McEachern, Kerry Anne, Chuang, Wei-Lien, Hutto, Elizabeth, Siegel, Craig S, Shayman, James A, Grabowski, Greg A, Scheule, Ronald K, Copeland, Diane P, Cheng, Seng H

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The chemosensitizing activity of inhibitors of glucosylceramide synthase is mediated primarily through modulation of P-gp function von CHAI, Lilly, MCLAREN, Rajashree P, TEICHER, Beverly A, CARTER, Kara, CHENG, Seng H, LEONARD, John P, YIBIN XIANG, VASCONCELLES, Michael, GOLDBERG, Mark A, COPELAND, Diane P, KLINGER, Katherine W, LILLIE, James, BYRNE, Ann, MADDEN, Stephen L, JIANG, Yide A, CHUANG, Wei-Lien, YINYIN HUANG, DUFAULT, Michael R, PACHECO, Joshua, MADHIWALLA, Shruti, XIAOKUI ZHANG, ZHANG, Mindy

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Inhibiting glycosphingolipid synthesis ameliorates hepatic steatosis in obese mice von Zhao, Hongmei, Przybylska, Malgorzata, Wu, I-Huan, Zhang, Jinhua, Maniatis, Panagiotis, Pacheco, Joshua, Piepenhagen, Peter, Copeland, Diane, Arbeeny, Cynthia, Shayman, James A., Aerts, Johannes M., Jiang, Canwen, Cheng, Seng H., Yew, Nelson S.

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Tumor necrosis factor in preterm and term labor von Romero, Roberto, Mazor, Moshe, Sepulveda, Waldo, Avila, Cecilia, Copeland, Diane, Williams, John

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A Glucosylceramide Synthase Inhibitor Prevents the Cytotoxic Effects of Shiga Toxin-2 on Human Renal Tubular Epithelial Cells von Silberstein, Claudia, Copeland, Diane P., Chiang, Wei-Lien, Repetto, Horacio A., Ibarra, Cristina

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Inhibiting glycosphingolipid synthesis ameliorates hepatic steatosis in obese mice von Zhao, Hongmei, Przybylska, Malgorzata, Wu, I‐Huan, Zhang, Jinhua, Maniatis, Panagiotis, Pacheco, Joshua, Piepenhagen, Peter, Copeland, Diane, Arbeeny, Cynthia, Shayman, James A., Aerts, Johannes M., Jiang, Canwen, Cheng, Seng H., Yew, Nelson S.

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Search for the optimal fetal cell antibody : results of immunophenotyping studies using flow cytometry von ZHENG, Y.-L, DONG KAI ZHEN, DEMARIA, M. A, BERRY, S. M, WAPNER, R. J, EVANS, M. I, COPELAND, D, WILLIAMS, J. M, BIANCHI, D. W

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90. Efficacy of Genz-529468-mediated inhibition of glucosylceramide synthase in a mouse model of Sandhoff disease von Marshall, John, Ashe, Karen, Li, Linyung, Nietupski, Jennifer, Aerts, Johannes, Copeland, Diane, Scheule, Ronald, Cheng, Seng

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91. Management of lysosomal glycosphingolipid levels in animal models of Gaucher and Fabry disease with enzyme and substrate reduction therapies von Marshall, John, Ashe, Karen, Chuang, WeiLien, Copeland, Diane, Scheule, Ronald, Cheng, Seng

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Measurement of lysosomal glucocerebrosidase activity in mouse liver using a fluorescence-activated cell sorter assay von Chan, Katherine Watt, Waire, James, Simons, Betsy, Karey, Ken, Fung, John, Copeland, Diane, Andrews, Laura

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Correction von Copeland, Diane

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Correction von Copeland, Diane

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Correction von Copeland, Diane

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