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Erik Bonten

Research Profile

Chemicals & Drugs

Disorders

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Amino Acid Sequences

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Publications

TOP 3
Huimin Hu; Arthur W Nienhuis; Jianrong Wu; Yanan Wu; Jim Allay; Erik J Bonten; Christopher Calabrese; Elida Gomero; John T Gray; Alessandra d'Azzo
Preclinical dose-finding study with a liver-tropic, recombinant AAV-2/8 vector in the mouse model of galactosialidosis.
Alessandra d'Azzo; Erik J Bonten
Molecular mechanisms of pathogenesis in a glycosphingolipid and a glycoprotein storage disease.
Nicholas M Stamatos; Ivan Carubelli; Diantha van de Vlekkert; Erik J Bonten; Nadia Papini; Chiguang Feng; Bruno Venerando; Alessandra d'Azzo; Alan S Cross; Lai-Xi Wang; Peter J Gomatos
LPS-induced cytokine production in human dendritic cells is regulated by sialidase activity.

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All Publications

2012: Huimin Hu; Arthur W Nienhuis; Jianrong Wu; Yanan Wu; Jim Allay; Erik J Bonten; Christopher Calabrese; Elida Gomero; John T Gray; Alessandra d'Azzo
Preclinical dose-finding study with a liver-tropic, recombinant AAV-2/8 vector in the mouse model of galactosialidosis.
Molecular therapy : the journal of the American Society of Gene Therapy 2012;20(2):267-74.
2010: Alessandra d'Azzo; Erik J Bonten
Molecular mechanisms of pathogenesis in a glycosphingolipid and a glycoprotein storage disease.
Biochemical Society transactions 2010;38(6):1453-7.
2010: Nicholas M Stamatos; Ivan Carubelli; Diantha van de Vlekkert; Erik J Bonten; Nadia Papini; Chiguang Feng; Bruno Venerando; Alessandra d'Azzo; Alan S Cross; Lai-Xi Wang; Peter J Gomatos
LPS-induced cytokine production in human dendritic cells is regulated by sialidase activity.
Journal of leukocyte biology 2010;88(6):1227-39.
2010: Edmar Zanoteli; Diantha van de Vlekkert; Erik J Bonten; Huimin Hu; Linda Mann; Elida M Gomero; A John Harris; Giulio Ghersi; Alessandra d'Azzo
Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue.
Biochimica et biophysica acta 2010;1802(7-8):659-72.
2010: Xudong Wu; Katherine A Steigelman; Erik J Bonten; Huimin Hu; Wenxuan He; Tianying Ren; Jian Zuo; Alessandra d'Azzo
Vacuolization and alterations of lysosomal membrane proteins in cochlear marginal cells contribute to hearing loss in neuraminidase 1-deficient mice.
Biochimica et biophysica acta 2010;1802(2):259-68.
2009: Dongning Wang; Slava Zaitsev; Garry Taylor; Alessandra d'Azzo; Erik J Bonten
Protective protein/cathepsin A rescues N-glycosylation defects in neuraminidase-1.
Biochimica et biophysica acta 2009;1790(4):275-82.
2008: Gouri Yogalingam; Erik J Bonten; Diantha van de Vlekkert; Huimin Hu; Simon Moshiach; Samuel A Connell; Alessandra d'Azzo
Neuraminidase 1 is a negative regulator of lysosomal exocytosis.
Developmental cell 2008;15(1):74-86.
2005: Dongning Wang; Erik J Bonten; Gouri Yogalingam; Linda Mann; Alessandra d'Azzo
Short-term, high dose enzyme replacement therapy in sialidosis mice.
Molecular genetics and metabolism 2005;85(3):181-9.
2004: Erik J Bonten; Dongning Wang; James N Toy; Linda Mann; Aurélie Mignardot; Gouri Yogalingam; Alessandra d'Azzo
Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2004;18(9):971-3.
2003: Ana Maria Cuervo; Linda Mann; Erik J Bonten; Alessandra d'Azzo; J Fred Dice
Cathepsin A regulates chaperone-mediated autophagy through cleavage of the lysosomal receptor.
The EMBO journal 2003;22(1):47-59.
2002: Natalie de Geest; Erik J Bonten; Linda Mann; Jean de Sousa-Hitzler; Chris Hahn; Alessandra d'Azzo
Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice.
Human molecular genetics 2002;11(12):1455-64.
2002: Thasia Leimig; Linda Mann; Maria del Pilar Martin; Erik J Bonten; Derek A Persons; James Knowles; James A Allay; John Cunningham; Arthur W Nienhuis; Richard Smeyne; Alessandra d'Azzo
Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells.
Blood 2002;99(9):3169-78.
2000: Erik J Bonten; Alessandra d'Azzo
Lysosomal neuraminidase. Catalytic activation in insect cells is controlled by the protective protein/cathepsin A.
The Journal of biological chemistry 2000;275(48):37657-63.
2000: Erik J Bonten; W F Arts; M Beck; A Covanis; Maria Alice Donati; Rossella Parini; Enrico Zammarchi; Alessandra d'Azzo
Novel mutations in lysosomal neuraminidase identify functional domains and determine clinical severity in sialidosis.
Human molecular genetics 2000;9(18):2715-25.
2000: Aarnoud C van der Spoel; Erik J Bonten; Alessandra d'Azzo
Processing of lysosomal beta-galactosidase. The C-terminal precursor fragment is an essential domain of the mature enzyme.
The Journal of biological chemistry 2000;275(14):10035-40.
1999: HL Tai; Michael Fessing; Erik J Bonten; Y Yanishevsky; Alessandra d'Azzo; Eugene Y Krynetski; William Evans
Enhanced proteasomal degradation of mutant human thiopurine S-methyltransferase (TPMT) in mammalian cells: mechanism for TPMT protein deficiency inherited by TPMT*2, TPMT*3A, TPMT*3B or TPMT*3C.
Pharmacogenetics 1999;9(5):641-50.
1998: Aarnoud C van der Spoel; Erik J Bonten; Alessandra d'Azzo
Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A.
The EMBO journal 1998;17(6):1588-97.
1998: Robbert Rottier; Erik J Bonten; Alessandra d'Azzo
A point mutation in the neu-1 locus causes the neuraminidase defect in the SM/J mouse.
Human molecular genetics 1998;7(2):313-21.
1998: Gabby Rudenko; Erik J Bonten; Wim G J Hol; Alessandra d'Azzo
The atomic model of the human protective protein/cathepsin A suggests a structural basis for galactosialidosis.
Proceedings of the National Academy of Sciences of the United States of America 1998;95(2):621-5.
1996: Erik J Bonten; A van der Spoel; Maarten Fornerod; Gerard C Grosveld; Alessandra d'Azzo
Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.
Genes & development 1996;10(24):3156-69.
1996: Gabby Rudenko; Erik J Bonten; Alessandra d'Azzo; Wim G J Hol
Structure determination of the human protective protein: twofold averaging reveals the three-dimensional structure of a domain which was entirely absent in the initial model.
Acta crystallographica. Section D, Biological crystallography 1996;52(Pt 5):923-36.
1995: Gabby Rudenko; Erik J Bonten; Alessandra d'Azzo; Wim G J Hol
Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism.
Structure (London, England : 1993) 1995;3(11):1249-59.
1995: Erik J Bonten; Niels Galjart; Rob Willemsen; M Usmany; Just M Vlak; Alessandra d'Azzo
Lysosomal protective protein/cathepsin A. Role of the "linker" domain in catalytic activation.
The Journal of biological chemistry 1995;270(44):26441-5.
1995: X Y Zhou; Hans Morreau; Robbert Rottier; D Davis; Erik J Bonten; Nynke Gillemans; D Wenger; Frank Grosveld; Peter C Doherty; K Suzuki; G C Grosveld; A d'Azzo
Mouse model for the lysosomal disorder galactosialidosis and correction of the phenotype with overexpressing erythroid precursor cells.
Genes & development 1995;9(21):2623-34.
1991: Hans Morreau; Erik J Bonten; X Y Zhou; Alessandra d'Azzo
Organization of the gene encoding human lysosomal beta-galactosidase.
DNA and cell biology 1991;10(7):495-504.
1991: Niels Galjart; Hans Morreau; Rob Willemsen; Nynke Gillemans; Erik J Bonten; Alessandra d'Azzo
Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function.
The Journal of biological chemistry 1991;266(22):14754-62.