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Items: 22


Inflammasome-mediated glucocorticoid resistance: The receptor rheostat.

Paugh SW, Bonten EJ, Evans WE.

Mol Cell Oncol. 2015 Nov 11;3(1):e1065947. doi: 10.1080/23723556.2015.1065947. eCollection 2016 Jan.


MicroRNAs Form Triplexes with Double Stranded DNA at Sequence-Specific Binding Sites; a Eukaryotic Mechanism via which microRNAs Could Directly Alter Gene Expression.

Paugh SW, Coss DR, Bao J, Laudermilk LT, Grace CR, Ferreira AM, Waddell MB, Ridout G, Naeve D, Leuze M, LoCascio PF, Panetta JC, Wilkinson MR, Pui CH, Naeve CW, Uberbacher EC, Bonten EJ, Evans WE.

PLoS Comput Biol. 2016 Feb 4;12(2):e1004744. doi: 10.1371/journal.pcbi.1004744. eCollection 2016 Feb.


NALP3 inflammasome upregulation and CASP1 cleavage of the glucocorticoid receptor cause glucocorticoid resistance in leukemia cells.

Paugh SW, Bonten EJ, Savic D, Ramsey LB, Thierfelder WE, Gurung P, Malireddi RK, Actis M, Mayasundari A, Min J, Coss DR, Laudermilk LT, Panetta JC, McCorkle JR, Fan Y, Crews KR, Stocco G, Wilkinson MR, Ferreira AM, Cheng C, Yang W, Karol SE, Fernandez CA, Diouf B, Smith C, Hicks JK, Zanut A, Giordanengo A, Crona D, Bianchi JJ, Holmfeldt L, Mullighan CG, den Boer ML, Pieters R, Jeha S, Dunwell TL, Latif F, Bhojwani D, Carroll WL, Pui CH, Myers RM, Guy RK, Kanneganti TD, Relling MV, Evans WE.

Nat Genet. 2015 Jun;47(6):607-14. doi: 10.1038/ng.3283. Epub 2015 May 4.


Lysosomal multienzyme complex: pros and cons of working together.

Bonten EJ, Annunziata I, d'Azzo A.

Cell Mol Life Sci. 2014 Jun;71(11):2017-32. doi: 10.1007/s00018-013-1538-3. Epub 2013 Dec 15. Review.


Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis.

Bonten EJ, Yogalingam G, Hu H, Gomero E, van de Vlekkert D, d'Azzo A.

Biochim Biophys Acta. 2013 Oct;1832(10):1784-92. doi: 10.1016/j.bbadis.2013.06.002. Epub 2013 Jun 12.


LPS-induced cytokine production in human dendritic cells is regulated by sialidase activity.

Stamatos NM, Carubelli I, van de Vlekkert D, Bonten EJ, Papini N, Feng C, Venerando B, d'Azzo A, Cross AS, Wang LX, Gomatos PJ.

J Leukoc Biol. 2010 Dec;88(6):1227-39. doi: 10.1189/jlb.1209776. Epub 2010 Sep 8.


Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue.

Zanoteli E, van de Vlekkert D, Bonten EJ, Hu H, Mann L, Gomero EM, Harris AJ, Ghersi G, d'Azzo A.

Biochim Biophys Acta. 2010 Jul-Aug;1802(7-8):659-72. doi: 10.1016/j.bbadis.2010.04.002. Epub 2010 Apr 11.


Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization.

Bonten EJ, Campos Y, Zaitsev V, Nourse A, Waddell B, Lewis W, Taylor G, d'Azzo A.

J Biol Chem. 2009 Oct 9;284(41):28430-41. doi: 10.1074/jbc.M109.031419. Epub 2009 Aug 7.


Neuraminidase 1 is a negative regulator of lysosomal exocytosis.

Yogalingam G, Bonten EJ, van de Vlekkert D, Hu H, Moshiach S, Connell SA, d'Azzo A.

Dev Cell. 2008 Jul;15(1):74-86. doi: 10.1016/j.devcel.2008.05.005.


Short-term, high dose enzyme replacement therapy in sialidosis mice.

Wang D, Bonten EJ, Yogalingam G, Mann L, d'Azzo A.

Mol Genet Metab. 2005 Jul;85(3):181-9. Epub 2005 Apr 25.


Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis.

Bonten EJ, Wang D, Toy JN, Mann L, Mignardot A, Yogalingam G, D'Azzo A.

FASEB J. 2004 Jun;18(9):971-3. Epub 2004 Apr 14.


Cathepsin A regulates chaperone-mediated autophagy through cleavage of the lysosomal receptor.

Cuervo AM, Mann L, Bonten EJ, d'Azzo A, Dice JF.

EMBO J. 2003 Jan 2;22(1):47-59.


Novel mutations in lysosomal neuraminidase identify functional domains and determine clinical severity in sialidosis.

Bonten EJ, Arts WF, Beck M, Covanis A, Donati MA, Parini R, Zammarchi E, d'Azzo A.

Hum Mol Genet. 2000 Nov 1;9(18):2715-25.


Lysosomal protective protein/cathepsin A. Role of the "linker" domain in catalytic activation.

Bonten EJ, Galjart NJ, Willemsen R, Usmany M, Vlak JM, d'Azzo A.

J Biol Chem. 1995 Nov 3;270(44):26441-5.


Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function.

Galjart NJ, Morreau H, Willemsen R, Gillemans N, Bonten EJ, d'Azzo A.

J Biol Chem. 1991 Aug 5;266(22):14754-62.


Germinal mosaicism increases the recurrence risk for 'new' Duchenne muscular dystrophy mutations.

Bakker E, Veenema H, Den Dunnen JT, van Broeckhoven C, Grootscholten PM, Bonten EJ, van Ommen GJ, Pearson PL.

J Med Genet. 1989 Sep;26(9):553-9.


Carrier detection and prenatal diagnosis of Duchenne/Becker muscular dystrophy (D/BMD) by DNA-analysis.

Bakker E, Bonten EJ, den Dunnen JT, Veenema H, Grootscholten PM, van Ommen GJ, Pearson PL.

Prog Clin Biol Res. 1989;306:51-67. Review. No abstract available.


Prenatal diagnosis of Duchenne muscular dystrophy: a three-year experience in a rapidly evolving field.

Bakker E, Bonten EJ, Veenema H, den Dunnen JT, Grootscholten PM, van Ommen GJ, Pearson PL.

J Inherit Metab Dis. 1989;12 Suppl 1:174-90. Review.


Germline mosaicism and Duchenne muscular dystrophy mutations.

Bakker E, Van Broeckhoven C, Bonten EJ, van de Vooren MJ, Veenema H, Van Hul W, Van Ommen GJ, Vandenberghe A, Pearson PL.

Nature. 1987 Oct 8-14;329(6139):554-6.


DNA probe analysis for carrier detection and prenatal diagnosis of Duchenne muscular dystrophy: a standard diagnostic procedure.

Bakker E, Bonten EJ, De Lange LF, Veenema H, Majoor-Krakauer D, Hofker MH, Van Ommen GJ, Pearson PL.

J Med Genet. 1986 Dec;23(6):573-80.

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