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Items: 42


Selective inhibition of N-linked glycosylation impairs receptor tyrosine kinase processing.

Klaver E, Zhao P, May M, Flanagan-Steet H, Freeze HH, Gilmore R, Wells L, Contessa J, Steet R.

Dis Model Mech. 2019 Jun 5;12(6). pii: dmm039602. doi: 10.1242/dmm.039602.


Defective mucin-type glycosylation on α-dystroglycan in COG-deficient cells increases its susceptibility to bacterial proteases.

Yu SH, Zhao P, Prabhakar PK, Sun T, Beedle A, Boons GJ, Moremen KW, Wells L, Steet R.

J Biol Chem. 2018 Sep 14;293(37):14534-14544. doi: 10.1074/jbc.RA118.003014. Epub 2018 Jul 26.


Site-1 protease deficiency causes human skeletal dysplasia due to defective inter-organelle protein trafficking.

Kondo Y, Fu J, Wang H, Hoover C, McDaniel JM, Steet R, Patra D, Song J, Pollard L, Cathey S, Yago T, Wiley G, Macwana S, Guthridge J, McGee S, Li S, Griffin C, Furukawa K, James JA, Ruan C, McEver RP, Wierenga KJ, Gaffney PM, Xia L.

JCI Insight. 2018 Jul 26;3(14). pii: 121596. doi: 10.1172/jci.insight.121596. eCollection 2018 Jul 26.


TGF-ß Regulates Cathepsin Activation during Normal and Pathogenic Development.

Flanagan-Steet H, Christian C, Lu PN, Aarnio-Peterson M, Sanman L, Archer-Hartmann S, Azadi P, Bogyo M, Steet RA.

Cell Rep. 2018 Mar 13;22(11):2964-2977. doi: 10.1016/j.celrep.2018.02.066.


Altered Met receptor phosphorylation and LRP1-mediated uptake in cells lacking carbohydrate-dependent lysosomal targeting.

Aarnio-Peterson M, Zhao P, Yu SH, Christian C, Flanagan-Steet H, Wells L, Steet R.

J Biol Chem. 2017 Sep 8;292(36):15094-15104. doi: 10.1074/jbc.M117.790139. Epub 2017 Jul 19.


Glycosylation and stem cells: Regulatory roles and application of iPSCs in the study of glycosylation-related disorders.

Berger RP, Dookwah M, Steet R, Dalton S.

Bioessays. 2016 Dec;38(12):1255-1265. doi: 10.1002/bies.201600138. Epub 2016 Sep 26. Review.


One-Step Selective Exoenzymatic Labeling (SEEL) Strategy for the Biotinylation and Identification of Glycoproteins of Living Cells.

Sun T, Yu SH, Zhao P, Meng L, Moremen KW, Wells L, Steet R, Boons GJ.

J Am Chem Soc. 2016 Sep 14;138(36):11575-11582. doi: 10.1021/jacs.6b04049. Epub 2016 Sep 1.


Enzyme-specific differences in mannose phosphorylation between GlcNAc-1-phosphotransferase αβ and γ subunit deficient zebrafish support cathepsin proteases as early mediators of mucolipidosis pathology.

Flanagan-Steet H, Matheny C, Petrey A, Parker J, Steet R.

Biochim Biophys Acta. 2016 Sep;1860(9):1845-53. doi: 10.1016/j.bbagen.2016.05.029. Epub 2016 May 27.


Selective Exo-Enzymatic Labeling Detects Increased Cell Surface Sialoglycoprotein Expression upon Megakaryocytic Differentiation.

Yu SH, Zhao P, Sun T, Gao Z, Moremen KW, Boons GJ, Wells L, Steet R.

J Biol Chem. 2016 Feb 19;291(8):3982-9. doi: 10.1074/jbc.M115.700369. Epub 2016 Jan 5.


Cyclopropenone-caged Sondheimer diyne (dibenzo[a,e]cyclooctadiyne): a photoactivatable linchpin for efficient SPAAC crosslinking.

Sutton DA, Yu SH, Steet R, Popik VV.

Chem Commun (Camb). 2016 Jan 11;52(3):553-6. doi: 10.1039/c5cc08106h. Epub 2015 Nov 5.


A beta-blocker, propranolol, decreases the efficacy from enzyme replacement therapy in Pompe disease.

Han SO, Pope R, Li S, Kishnani PS, Steet R, Koeberl DD.

Mol Genet Metab. 2016 Feb;117(2):114-9. doi: 10.1016/j.ymgme.2015.09.012. Epub 2015 Oct 3.


Evaluation of AAV-mediated Gene Therapy for Central Nervous System Disease in Canine Mucopolysaccharidosis VII.

Gurda BL, De Guilhem De Lataillade A, Bell P, Zhu Y, Yu H, Wang P, Bagel J, Vite CH, Sikora T, Hinderer C, Calcedo R, Yox AD, Steet RA, Ruane T, O'Donnell P, Gao G, Wilson JM, Casal M, Ponder KP, Haskins ME.

Mol Ther. 2016 Feb;24(2):206-216. doi: 10.1038/mt.2015.189. Epub 2015 Oct 8.


Cathepsin-Mediated Alterations in TGFß-Related Signaling Underlie Disrupted Cartilage and Bone Maturation Associated With Impaired Lysosomal Targeting.

Flanagan-Steet H, Aarnio M, Kwan B, Guihard P, Petrey A, Haskins M, Blanchard F, Steet R.

J Bone Miner Res. 2016 Mar;31(3):535-48. doi: 10.1002/jbmr.2722. Epub 2015 Oct 13.


Abnormal cartilage development and altered N-glycosylation in Tmem165-deficient zebrafish mirrors the phenotypes associated with TMEM165-CDG.

Bammens R, Mehta N, Race V, Foulquier F, Jaeken J, Tiemeyer M, Steet R, Matthijs G, Flanagan-Steet H.

Glycobiology. 2015 Jun;25(6):669-82. doi: 10.1093/glycob/cwv009. Epub 2015 Jan 21.


Analysis of mucolipidosis II/III GNPTAB missense mutations identifies domains of UDP-GlcNAc:lysosomal enzyme GlcNAc-1-phosphotransferase involved in catalytic function and lysosomal enzyme recognition.

Qian Y, van Meel E, Flanagan-Steet H, Yox A, Steet R, Kornfeld S.

J Biol Chem. 2015 Jan 30;290(5):3045-56. doi: 10.1074/jbc.M114.612507. Epub 2014 Dec 11.


Selective exo-enzymatic labeling of N-glycans on the surface of living cells by recombinant ST6Gal I.

Mbua NE, Li X, Flanagan-Steet HR, Meng L, Aoki K, Moremen KW, Wolfert MA, Steet R, Boons GJ.

Angew Chem Int Ed Engl. 2013 Dec 2;52(49):13012-5. doi: 10.1002/anie.201307095. Epub 2013 Oct 15.


A novel intermediate mucolipidosis II/IIIαβ caused by GNPTAB mutation in the cytosolic N-terminal domain.

Leroy JG, Sillence D, Wood T, Barnes J, Lebel RR, Friez MJ, Stevenson RE, Steet R, Cathey SS.

Eur J Hum Genet. 2014 May;22(5):594-601. doi: 10.1038/ejhg.2013.207. Epub 2013 Sep 18.


A mutation in a ganglioside biosynthetic enzyme, ST3GAL5, results in salt & pepper syndrome, a neurocutaneous disorder with altered glycolipid and glycoprotein glycosylation.

Boccuto L, Aoki K, Flanagan-Steet H, Chen CF, Fan X, Bartel F, Petukh M, Pittman A, Saul R, Chaubey A, Alexov E, Tiemeyer M, Steet R, Schwartz CE.

Hum Mol Genet. 2014 Jan 15;23(2):418-33. doi: 10.1093/hmg/ddt434. Epub 2013 Sep 10.


Abnormal accumulation and recycling of glycoproteins visualized in Niemann-Pick type C cells using the chemical reporter strategy.

Mbua NE, Flanagan-Steet H, Johnson S, Wolfert MA, Boons GJ, Steet R.

Proc Natl Acad Sci U S A. 2013 Jun 18;110(25):10207-12. doi: 10.1073/pnas.1221105110. Epub 2013 Jun 3.


The DMAP interaction domain of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase is a substrate recognition module.

Qian Y, Flanagan-Steet H, van Meel E, Steet R, Kornfeld SA.

Proc Natl Acad Sci U S A. 2013 Jun 18;110(25):10246-51. doi: 10.1073/pnas.1308453110. Epub 2013 Jun 3.


A zebrafish model of PMM2-CDG reveals altered neurogenesis and a substrate-accumulation mechanism for N-linked glycosylation deficiency.

Cline A, Gao N, Flanagan-Steet H, Sharma V, Rosa S, Sonon R, Azadi P, Sadler KC, Freeze HH, Lehrman MA, Steet R.

Mol Biol Cell. 2012 Nov;23(21):4175-87. doi: 10.1091/mbc.E12-05-0411. Epub 2012 Sep 5.


A zebrafish model of congenital disorders of glycosylation with phosphomannose isomerase deficiency reveals an early opportunity for corrective mannose supplementation.

Chu J, Mir A, Gao N, Rosa S, Monson C, Sharma V, Steet R, Freeze HH, Lehrman MA, Sadler KC.

Dis Model Mech. 2013 Jan;6(1):95-105. doi: 10.1242/dmm.010116. Epub 2012 Aug 16.


"Casting" light on the role of glycosylation during embryonic development: insights from zebrafish.

Flanagan-Steet HR, Steet R.

Glycoconj J. 2013 Jan;30(1):33-40. doi: 10.1007/s10719-012-9390-5. Epub 2012 May 26. Review.


Polar dibenzocyclooctynes for selective labeling of extracellular glycoconjugates of living cells.

Friscourt F, Ledin PA, Mbua NE, Flanagan-Steet HR, Wolfert MA, Steet R, Boons GJ.

J Am Chem Soc. 2012 Mar 21;134(11):5381-9. doi: 10.1021/ja3002666. Epub 2012 Mar 9.


Latency-associated peptide of transforming growth factor-β1 is not subject to physiological mannose phosphorylation.

Barnes J, Warejcka D, Simpliciano J, Twining S, Steet R.

J Biol Chem. 2012 Mar 2;287(10):7526-34. doi: 10.1074/jbc.M111.308825. Epub 2012 Jan 18.


Excessive activity of cathepsin K is associated with cartilage defects in a zebrafish model of mucolipidosis II.

Petrey AC, Flanagan-Steet H, Johnson S, Fan X, De la Rosa M, Haskins ME, Nairn AV, Moremen KW, Steet R.

Dis Model Mech. 2012 Mar;5(2):177-90. doi: 10.1242/dmm.008219. Epub 2011 Nov 1.


Mislocalization of large ARF-GEFs as a potential mechanism for BFA resistance in COG-deficient cells.

Flanagan-Steet H, Johnson S, Smith RD, Bangiyeva J, Lupashin V, Steet R.

Exp Cell Res. 2011 Oct 1;317(16):2342-52. doi: 10.1016/j.yexcr.2011.06.005. Epub 2011 Jun 22.


Strain-promoted alkyne-azide cycloadditions (SPAAC) reveal new features of glycoconjugate biosynthesis.

Mbua NE, Guo J, Wolfert MA, Steet R, Boons GJ.

Chembiochem. 2011 Aug 16;12(12):1912-21. doi: 10.1002/cbic.201100117. Epub 2011 Jun 9.


Extensive mannose phosphorylation on leukemia inhibitory factor (LIF) controls its extracellular levels by multiple mechanisms.

Barnes J, Lim JM, Godard A, Blanchard F, Wells L, Steet R.

J Biol Chem. 2011 Jul 15;286(28):24855-64. doi: 10.1074/jbc.M111.221432. Epub 2011 May 25.


α-Galactosidase A expressed in the salivary glands partially corrects organ biochemical deficits in the fabry mouse through endocrine trafficking.

Passineau MJ, Fahrenholz T, Machen L, Zourelias L, Nega K, Paul R, MacDougall MJ, Mamaeva O, Steet R, Barnes J, Kingston HM, Benza RL.

Hum Gene Ther. 2011 Mar;22(3):293-301. doi: 10.1089/hum.2010.069. Epub 2011 Jan 27.


Selective yolk deposition and mannose phosphorylation of lysosomal glycosidases in zebrafish.

Fan X, Klein M, Flanagan-Steet HR, Steet R.

J Biol Chem. 2010 Oct 22;285(43):32946-53. doi: 10.1074/jbc.M110.158295. Epub 2010 Aug 20.


Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II.

Flanagan-Steet H, Sias C, Steet R.

Am J Pathol. 2009 Nov;175(5):2063-75. doi: 10.2353/ajpath.2009.090210. Epub 2009 Oct 15.


Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase.

Steet R, Chung S, Lee WS, Pine CW, Do H, Kornfeld S.

Biochem Pharmacol. 2007 May 1;73(9):1376-83. Epub 2006 Dec 15.


The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.

Steet RA, Chung S, Wustman B, Powe A, Do H, Kornfeld SA.

Proc Natl Acad Sci U S A. 2006 Sep 12;103(37):13813-8. Epub 2006 Aug 31.


COG-7-deficient Human Fibroblasts Exhibit Altered Recycling of Golgi Proteins.

Steet R, Kornfeld S.

Mol Biol Cell. 2006 May;17(5):2312-21. Epub 2006 Mar 1.


Clinical and biochemical presentation of siblings with COG-7 deficiency, a lethal multiple O- and N-glycosylation disorder.

Spaapen LJ, Bakker JA, van der Meer SB, Sijstermans HJ, Steet RA, Wevers RA, Jaeken J.

J Inherit Metab Dis. 2005;28(5):707-14.


Identification of the minimal lysosomal enzyme recognition domain in cathepsin D.

Steet R, Lee WS, Kornfeld S.

J Biol Chem. 2005 Sep 30;280(39):33318-23. Epub 2005 Aug 4.


A splicing mutation in the alpha/beta GlcNAc-1-phosphotransferase gene results in an adult onset form of mucolipidosis III associated with sensory neuropathy and cardiomyopathy.

Steet RA, Hullin R, Kudo M, Martinelli M, Bosshard NU, Schaffner T, Kornfeld S, Steinmann B.

Am J Med Genet A. 2005 Feb 1;132A(4):369-75.


Mutation of the COG complex subunit gene COG7 causes a lethal congenital disorder.

Wu X, Steet RA, Bohorov O, Bakker J, Newell J, Krieger M, Spaapen L, Kornfeld S, Freeze HH.

Nat Med. 2004 May;10(5):518-23. Epub 2004 Apr 25.


3'-Azidothymidine (zidovudine) inhibits glycosylation and dramatically alters glycosphingolipid synthesis in whole cells at clinically relevant concentrations.

Yan JP, Ilsley DD, Frohlick C, Steet R, Hall ET, Kuchta RD, Melançon P.

J Biol Chem. 1995 Sep 29;270(39):22836-41.

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