| Year |
Citation |
Score |
| 2023 |
Dookwah M, Wagner SK, Ishihara M, Yu SH, Ulrichs H, Kulik MJ, Zeltner N, Dalton S, Strauss KA, Aoki K, Steet R, Tiemeyer M. Neural-specific alterations in glycosphingolipid biosynthesis and cell signaling associated with two human ganglioside GM3 synthase deficiency variants. Human Molecular Genetics. PMID 37676252 DOI: 10.1093/hmg/ddad146 |
0.334 |
|
| 2022 |
Tao X, Zhu Y, Diaz-Perez Z, Yu SH, Foley JR, Stewart TM, Casero RA, Steet R, Zhai RG. Phenylbutyrate modulates polyamine acetylase and ameliorates Snyder-Robinson syndrome in a Drosophila model and patient cells. Jci Insight. 7. PMID 35801587 DOI: 10.1172/jci.insight.158457 |
0.307 |
|
| 2021 |
Klaver EJ, Dukes-Rimsky L, Kumar B, Xia ZJ, Dang T, Lehrman MA, Angel P, Drake RR, Freeze HH, Steet R, Flanagan-Steet H. Protease-dependent defects in N-cadherin processing drive PMM2-CDG pathogenesis. Jci Insight. PMID 34784297 DOI: 10.1172/jci.insight.153474 |
0.317 |
|
| 2021 |
Ng BG, Sosicka P, Fenaille F, Harroche A, Vuillaumier-Barrot S, Porterfield M, Xia ZJ, Wagner S, Bamshad MJ, Vergnes-Boiteux MC, Cholet S, Dalton S, Dell A, Dupré T, Fiore M, ... ... Steet R, et al. A mutation in SLC37A4 causes a dominantly inherited congenital disorder of glycosylation characterized by liver dysfunction. American Journal of Human Genetics. PMID 33964207 DOI: 10.1016/j.ajhg.2021.04.013 |
0.308 |
|
| 2019 |
Klaver E, Zhao P, May M, Flanagan-Steet H, Freeze HH, Gilmore R, Wells L, Contessa J, Steet R. Selective inhibition of N-linked glycosylation impairs receptor tyrosine kinase processing. Disease Models & Mechanisms. PMID 31101650 DOI: 10.1242/Dmm.039602 |
0.38 |
|
| 2016 |
Yu SH, Zhao P, Sun T, Gao Z, Moremen KW, Boons GJ, Wells L, Steet R. Selective exo-enzymatic labeling detects increased cell surface sialoglycoprotein expression upon megakaryocytic differentiation. The Journal of Biological Chemistry. PMID 26733198 DOI: 10.1074/Jbc.M115.700369 |
0.341 |
|
| 2013 |
Mbua NE, Li X, Flanagan-Steet HR, Meng L, Aoki K, Moremen KW, Wolfert MA, Steet R, Boons GJ. Selective exo-enzymatic labeling of N-glycans on the surface of living cells by recombinant ST6Gal I. Angewandte Chemie (International Ed. in English). 52: 13012-5. PMID 24129959 DOI: 10.1002/Anie.201307095 |
0.337 |
|
| 2013 |
Mbua NE, Flanagan-Steet H, Johnson S, Wolfert MA, Boons GJ, Steet R. Abnormal accumulation and recycling of glycoproteins visualized in Niemann-Pick type C cells using the chemical reporter strategy. Proceedings of the National Academy of Sciences of the United States of America. 110: 10207-12. PMID 23733943 DOI: 10.1073/Pnas.1221105110 |
0.367 |
|
| 2012 |
Cline A, Gao N, Flanagan-Steet H, Sharma V, Rosa S, Sonon R, Azadi P, Sadler KC, Freeze HH, Lehrman MA, Steet R. A zebrafish model of PMM2-CDG reveals altered neurogenesis and a substrate-accumulation mechanism for N-linked glycosylation deficiency. Molecular Biology of the Cell. 23: 4175-87. PMID 22956764 DOI: 10.1091/Mbc.E12-05-0411 |
0.308 |
|
| 2007 |
Steet R, Chung S, Lee WS, Pine CW, Do H, Kornfeld S. Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase. Biochemical Pharmacology. 73: 1376-83. PMID 17217920 DOI: 10.1016/J.Bcp.2006.12.015 |
0.351 |
|
| 2006 |
Steet RA, Chung S, Wustman B, Powe A, Do H, Kornfeld SA. The iminosugar isofagomine increases the activity of N370S mutant acid β-glucosidase in Gaucher fibroblasts by several mechanisms Proceedings of the National Academy of Sciences of the United States of America. 103: 13813-13818. PMID 16945909 DOI: 10.1073/Pnas.0605928103 |
0.338 |
|
| 1999 |
Steet R, Alizadeh M, Melançon P, Kuchta RD. 3'-Azidothymidine significantly alters glycosphingolipid synthesis in melanoma cells and decreases the shedding of gangliosides Glycoconjugate Journal. 16: 237-245. PMID 10596899 DOI: 10.1023/A:1007032522001 |
0.474 |
|
| 1995 |
Yan JP, Ilsley DD, Frohlick C, Steet R, Hall ET, Kuchta RD, Melançon P. 3′-Azidothymidine (Zidovudine) inhibits glycosylation and dramatically alters glycosphingolipid synthesis in whole cells at clinically relevant concentrations Journal of Biological Chemistry. 270: 22836-22841. PMID 7559416 DOI: 10.1074/Jbc.270.39.22836 |
0.395 |
|
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