Pacheco JJ - Search Results

1

Iminosugar-based inhibitors of glucosylceramide synthase prolong survival but paradoxically increase brain glucosylceramide levels in Niemann-Pick C mice.

Nietupski JB, Pacheco JJ, Chuang WL, Maratea K, Li L, Foley J, Ashe KM, Cooper CG, Aerts JM, Copeland DP, Scheule RK, Cheng SH, Marshall J. Nietupski JB, et al. Among authors: pacheco jj. Mol Genet Metab. 2012 Apr;105(4):621-8. doi: 10.1016/j.ymgme.2012.01.020. Epub 2012 Feb 1. Mol Genet Metab. 2012. PMID: 22366055

2

Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.

Marshall J, Ashe KM, Bangari D, McEachern K, Chuang WL, Pacheco J, Copeland DP, Desnick RJ, Shayman JA, Scheule RK, Cheng SH. Marshall J, et al. PLoS One. 2010 Nov 24;5(11):e15033. doi: 10.1371/journal.pone.0015033. PLoS One. 2010. PMID: 21124789 Free PMC article.

3

Lyso-sphingomyelin is elevated in dried blood spots of Niemann-Pick B patients.

Chuang WL, Pacheco J, Cooper S, McGovern MM, Cox GF, Keutzer J, Zhang XK. Chuang WL, et al. Mol Genet Metab. 2014 Feb;111(2):209-11. doi: 10.1016/j.ymgme.2013.11.012. Epub 2013 Dec 7. Mol Genet Metab. 2014. PMID: 24418695

4

Nonclinical safety assessment of recombinant human acid sphingomyelinase (rhASM) for the treatment of acid sphingomyelinase deficiency:the utility of animal models of disease in the toxicological evaluation of potential therapeutics.

Murray JM, Thompson AM, Vitsky A, Hawes M, Chuang WL, Pacheco J, Wilson S, McPherson JM, Thurberg BL, Karey KP, Andrews L. Murray JM, et al. Mol Genet Metab. 2015 Feb;114(2):217-25. doi: 10.1016/j.ymgme.2014.07.005. Epub 2014 Jul 12. Mol Genet Metab. 2015. PMID: 25092414 Free article.

5

Intracerebroventricular delivery of glucocerebrosidase reduces substrates and increases lifespan in a mouse model of neuronopathic Gaucher disease.

Cabrera-Salazar MA, Bercury SD, Ziegler RJ, Marshall J, Hodges BL, Chuang WL, Pacheco J, Li L, Cheng SH, Scheule RK. Cabrera-Salazar MA, et al. Exp Neurol. 2010 Oct;225(2):436-44. doi: 10.1016/j.expneurol.2010.07.023. Epub 2010 Jul 29. Exp Neurol. 2010. PMID: 20673762

6

Inhibiting glycosphingolipid synthesis ameliorates hepatic steatosis in obese mice.

Zhao H, Przybylska M, Wu IH, Zhang J, Maniatis P, Pacheco J, Piepenhagen P, Copeland D, Arbeeny C, Shayman JA, Aerts JM, Jiang C, Cheng SH, Yew NS. Zhao H, et al. Hepatology. 2009 Jul;50(1):85-93. doi: 10.1002/hep.22970. Hepatology. 2009. PMID: 19444873 Free article.

7

The chemosensitizing activity of inhibitors of glucosylceramide synthase is mediated primarily through modulation of P-gp function.

Chai L, McLaren RP, Byrne A, Chuang WL, Huang Y, Dufault MR, Pacheco J, Madhiwalla S, Zhang X, Zhang M, Teicher BA, Carter K, Cheng SH, Leonard JP, Xiang Y, Vasconcelles M, Goldberg MA, Copeland DP, Klinger KW, Lillie J, Madden SL, Jiang YA. Chai L, et al. Int J Oncol. 2011 Mar;38(3):701-11. doi: 10.3892/ijo.2010.888. Epub 2010 Dec 24. Int J Oncol. 2011. PMID: 21186402

8

Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis.

Dodge JC, Treleaven CM, Pacheco J, Cooper S, Bao C, Abraham M, Cromwell M, Sardi SP, Chuang WL, Sidman RL, Cheng SH, Shihabuddin LS. Dodge JC, et al. Proc Natl Acad Sci U S A. 2015 Jun 30;112(26):8100-5. doi: 10.1073/pnas.1508767112. Epub 2015 Jun 8. Proc Natl Acad Sci U S A. 2015. PMID: 26056266 Free PMC article.

9

A Simple, High-Throughput Method for Analysis of Ceramide, Glucosylceramide, and Ceramide Trihexoside in Dried Blood Spots by LC/MS/MS.

Chuang WL, Pacheco J, Zhang K. Chuang WL, et al. Methods Mol Biol. 2016;1378:263-72. doi: 10.1007/978-1-4939-3182-8_28. Methods Mol Biol. 2016. PMID: 26602138

10

Improved sensitivity of an acid sphingomyelinase activity assay using a C6:0 sphingomyelin substrate.

Chuang WL, Pacheco J, Cooper S, Kingsbury JS, Hinds J, Wolf P, Oliva P, Keutzer J, Cox GF, Zhang K. Chuang WL, et al. Mol Genet Metab Rep. 2015 Apr 17;3:55-7. doi: 10.1016/j.ymgmr.2015.04.001. eCollection 2015 Jun. Mol Genet Metab Rep. 2015. PMID: 26937397 Free PMC article.

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