Bonten E - Search Results

1

Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice.

de Geest N, Bonten E, Mann L, de Sousa-Hitzler J, Hahn C, d'Azzo A. de Geest N, et al. Among authors: bonten e. Hum Mol Genet. 2002 Jun 1;11(12):1455-64. doi: 10.1093/hmg/11.12.1455. Hum Mol Genet. 2002. PMID: 12023988

2

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. Bonten EJ, et al. FASEB J. 2004 Jun;18(9):971-3. doi: 10.1096/fj.03-0941fje. Epub 2004 Apr 14. FASEB J. 2004. PMID: 15084520

3

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

Wang D, Bonten EJ, Yogalingam G, Mann L, d'Azzo A. Wang D, et al. Mol Genet Metab. 2005 Jul;85(3):181-9. doi: 10.1016/j.ymgme.2005.03.007. Epub 2005 Apr 25. Mol Genet Metab. 2005. PMID: 15979029

4

Lysosomal multienzyme complex: pros and cons of working together.

Bonten EJ, Annunziata I, d'Azzo A. Bonten EJ, et al. Cell Mol Life Sci. 2014 Jun;71(11):2017-32. doi: 10.1007/s00018-013-1538-3. Epub 2013 Dec 15. Cell Mol Life Sci. 2014. PMID: 24337808 Free PMC article. Review.

5

Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells.

Leimig T, Mann L, Martin Mdel P, Bonten E, Persons D, Knowles J, Allay JA, Cunningham J, Nienhuis AW, Smeyne R, d'Azzo A. Leimig T, et al. Among authors: bonten e. Blood. 2002 May 1;99(9):3169-78. doi: 10.1182/blood.v99.9.3169. Blood. 2002. PMID: 11964280 Free article.

6

Molecular mechanisms of pathogenesis in a glycosphingolipid and a glycoprotein storage disease.

d'Azzo A, Bonten E. d'Azzo A, et al. Among authors: bonten e. Biochem Soc Trans. 2010 Dec;38(6):1453-7. doi: 10.1042/BST0381453. Biochem Soc Trans. 2010. PMID: 21118106 Free PMC article.

7

A point mutation in the neu-1 locus causes the neuraminidase defect in the SM/J mouse.

Rottier RJ, Bonten E, d'Azzo A. Rottier RJ, et al. Among authors: bonten e. Hum Mol Genet. 1998 Feb;7(2):313-21. doi: 10.1093/hmg/7.2.313. Hum Mol Genet. 1998. PMID: 9425240

8

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. Bonten EJ, et al. Hum Mol Genet. 2000 Nov 1;9(18):2715-25. doi: 10.1093/hmg/9.18.2715. Hum Mol Genet. 2000. PMID: 11063730

9

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. Yogalingam G, et al. Dev Cell. 2008 Jul;15(1):74-86. doi: 10.1016/j.devcel.2008.05.005. Dev Cell. 2008. PMID: 18606142 Free PMC article.

10

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. Zanoteli E, et al. Among authors: bonten ej. Biochim Biophys Acta. 2010 Jul-Aug;1802(7-8):659-72. doi: 10.1016/j.bbadis.2010.04.002. Epub 2010 Apr 11. Biochim Biophys Acta. 2010. PMID: 20388541 Free PMC article.

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