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E. Bonten · SciRah

Researcher profile · Semantic Scholar

E. Bonten

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E. Bonten — researcher profile on SciRah

h-index: 26
Works: 51
Citations: 2,617

Most-cited papers

Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.
E. Bonten, A. C. van der Spoel, Maarten Fornerod, G. Grosveld +1 more
1996Genes & Development307 citations Open access
Semantic Scholar
Cathepsin A regulates chaperone‐mediated autophagy through cleavage of the lysosomal receptor
A. M. Cuervo, L. Mann, E. Bonten, A. d'Azzo +1 more
2003EMBO Journal192 citations Open access
Semantic Scholar
Sialidases in vertebrates: a family of enzymes tailored for several cell functions.
E. Monti, E. Bonten, A. d'Azzo, R. Bresciani +4 more
2010Advances in Carbohydrate Chemistry and Biochemistry183 citations
Semantic Scholar
Neuraminidase 1 is a Negative Regulator of Lysosomal Exocytosis
G. Yogalingam, E. Bonten, D. van de Vlekkert, Huimin Hu +3 more
2008Developmental Cell181 citations Open access
Semantic Scholar
Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function.
N. Galjart, H. Morreau, R. Willemsen, N. Gillemans +2 more
1991Journal of Biological Chemistry143 citations Open access
Semantic Scholar
Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A
A. C. van der Spoel, E. Bonten, A. d'Azzo
1998EMBO Journal134 citations Open access
Semantic Scholar
Mouse model for the lysosomal disorder galactosialidosis and correction of the phenotype with overexpressing erythroid precursor cells.
X.-Y. Zhou, H. Morreau, R. Rottier, D. Davis +8 more
1995Genes & Development127 citations Open access
Semantic Scholar
Novel mutations in lysosomal neuraminidase identify functional domains and determine clinical severity in sialidosis.
E. Bonten, W. Arts, Michael Beck, A. Covanis +4 more
2000Human Molecular Genetics126 citations Open access
Semantic Scholar
Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice.
N. de Geest, E. Bonten, L. Mann, Jean de Sousa-Hitzler +2 more
2002Human Molecular Genetics116 citations Open access
Semantic Scholar
Lysosomal Multienzyme Complex: Pros and Cons of Working Together
E. Bonten, Ida Annunziata, A. d'Azzo
2013Cellular and Molecular Life Sciences113 citations Open access
Semantic Scholar
LPS‐induced cytokine production in human dendritic cells is regulated by sialidase activity
N. Stamatos, Ivan Carubelli, D. van de Vlekkert, E. Bonten +7 more
2010Journal of Leukocyte Biology104 citations Open access
Semantic Scholar
Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism.
G. Rudenko, E. Bonten, A. d'Azzo, W. Hol
1995Structure92 citations Open access
Semantic Scholar
Heterodimerization of the Sialidase NEU1 with the Chaperone Protective Protein/Cathepsin A Prevents Its Premature Oligomerization*
E. Bonten, Yvan Campos, V. Zaitsev, A. Nourse +4 more
2009Journal of Biological Chemistry79 citations Open access
Semantic Scholar
Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells.
T. Leimig, L. Mann, Maria del Pilar Martin, E. Bonten +7 more
2002Blood69 citations
Semantic Scholar
Integrative genomic analyses reveal mechanisms of glucocorticoid resistance in acute lymphoblastic leukemia
Robert J. Autry, S. Paugh, R. Carter, Lei Shi +37 more
2020Nature Cancer64 citations Open access
Semantic Scholar
Muscle Degeneration in Neuramindase 1 Deficient Mice Results from Infiltration of the Muscle Fibers by Expanded Connective Tissue
E. Zanoteli, D. van de Vlekkert, E. Bonten, Huimin Hu +5 more
2010Biochimica et Biophysica Acta52 citations Open access
Semantic Scholar
Molecular Mechanisms of Pathogenesis in a Glycosphigolipid and a Glycoprotein Storage Disease
A. d'Azzo, E. Bonten
2010Biochemical Society Transactions51 citations Open access
Semantic Scholar
Vacuolization and alterations of lysosomal membrane proteins in cochlear marginal cells contribute to hearing loss in neuraminidase 1 – deficient mice
Xudong Wu, Katherine Steigelman, E. Bonten, Huimin Hu +4 more
2009Biochimica et Biophysica Acta46 citations Open access
Semantic Scholar
Short-term, high dose enzyme replacement therapy in sialidosis mice.
Dong-Ning Wang, E. Bonten, G. Yogalingam, L. Mann +1 more
2005Molecular Genetics and Metabolism44 citations
Semantic Scholar
Processing of lysosomal beta-galactosidase. The C-terminal precursor fragment is an essential domain of the mature enzyme.
A. C. van der Spoel, E. Bonten, A. d'Azzo
2000Journal of Biological Chemistry44 citations Open access
Semantic Scholar
Lysosomal Protective Protein/Cathepsin A
E. Bonten, N. Galjart, R. Willemsen, M. Usmany +2 more
1995Journal of Biological Chemistry40 citations Open access
Semantic Scholar
Chaperone–Mediated Gene Therapy with Recombinant AAV-PPCA in a New Mouse Model of Type I Sialidosis
E. Bonten, G. Yogalingam, Huimin Hu, Elida Gomero +2 more
2013Biochimica et Biophysica Acta38 citations Open access
Semantic Scholar
Organization of the Gene Encoding Human Lysosomal β-Galactosidase
H. Morreau, E. Bonten, Xiao-Yan Zhou, A. d'Azzo
199137 citations
Semantic Scholar
Preclinical dose-finding study with a liver-tropic, recombinant AAV-2/8 vector in the mouse model of galactosialidosis.
Huimin Hu, Elida Gomero, E. Bonten, J. Gray +6 more
2012Molecular Therapy35 citations Open access
Semantic Scholar
Lysosomal Neuraminidase
E. Bonten, A. d'Azzo
2000Journal of Biological Chemistry29 citations Open access
Semantic Scholar
Lysosomal Neuraminidase CATALYTIC ACTIVATION IN INSECT CELLS IS CONTROLLED BY THE PROTECTIVE PROTEIN/CATHEPSIN A*
E. Bonten, A. d'Azzo
200027 citations
Semantic Scholar
Protective Protein/Cathepsin A Rescues N-glycosylation defects in Neuraminidase-1
Dong-Ning Wang, S. Zaitsev, G. Taylor, A. d'Azzo +1 more
2009Biochimica et Biophysica Acta26 citations Open access
Semantic Scholar
Hypomethylation of NLRP3 gene promoter discriminates glucocorticoid‐resistant from glucocorticoid‐sensitive idiopathic nephrotic syndrome patients
M. Lucafò, S. Granata, E. Bonten, R. McCorkle +12 more
2020Clinical and Translational Science25 citations Open access
Semantic Scholar
Pharmacogenomics of intracellular methotrexate polyglutamates in patients' leukemia cells in vivo.
E. Lopez-Lopez, Robert J. Autry, Colton A. Smith, Wen-Jian Yang +16 more
2020Journal of Clinical Investigation20 citations Open access
Semantic Scholar
Amino acid stress response genes promote L-asparaginase resistance in pediatric acute lymphoblastic leukemia
D. Ferguson, J. R. McCorkle, Kelly R. Barnett, E. Bonten +22 more
2021Blood Advances18 citations Open access
Semantic Scholar
Inflammasome-mediated glucocorticoid resistance: The receptor rheostat
S. Paugh, E. Bonten, W. Evans
2016Molecular & Cellular Oncology15 citations Open access
Semantic Scholar
Identification of small molecules that mitigate vincristine‐induced neurotoxicity while sensitizing leukemia cells to vincristine
B. Diouf, C. Wing, J. Panetta, D. Eddins +24 more
2021Clinical and Translational Science13 citations Open access
Semantic Scholar
PACSIN2 as a modulator of autophagy and mercaptopurine cytotoxicity: mechanisms in lymphoid and intestinal cells
G. Zudeh, R. Franca, M. Lucafò, E. Bonten +7 more
2023Life Science Alliance7 citations Open access
Semantic Scholar
Processing of Lysosomal β-Galactosidase
A. C. van der Spoel, E. Bonten, A. d'Azzo
2000Journal of Biological Chemistry6 citations Open access
Semantic Scholar
Organization of the gene encoding human lysosomal beta-galactosidase.
H. Morreau, E. Bonten, X.-Y. Zhou, A. d'Azzo
1991DNA and Cell Biology5 citations
Semantic Scholar
Biophysical and functional study of CRL5Ozz, a muscle specific ubiquitin ligase complex
Yvan Campos, A. Nourse, A. Tanwar, R. Kalathur +2 more
2022Scientific Reports3 citations Open access
Semantic Scholar
Defect in Protective Protein/Cathepsin A
A. d'Azzo, E. Bonten
20123 citations
Semantic Scholar
CASPorter: A Novel Inducible Human CASP1/NALP3/ASC Inflammasome Biosensor
Chan Zou, Jordan A. Beard, Guoping Yang, W. Evans +1 more
2022Journal of Inflammation Research2 citations Open access
Semantic Scholar
Defective bone marrow homing and engraftment in sialidosis mice
G. Yogalingam, E. Bonten, T. Walker, L. Mann +2 more
20032 citations
Semantic Scholar
G.P.19.09 The role of neuraminidase 1 in muscle atrophy
E. Zanoteli, E. Bonten, D. van de Vlekkert, L. Mann +1 more
20071 citations
Semantic Scholar
Plaque Assay of Recombinant Baculovirus Using Commercially Available Serum-Free Insect Cell Medium: Improved Plaque Morphology, Easy Detection, and cGMP Compatibility
E. Bonten, A. d'Azzo
20061 citations
Semantic Scholar
Lysosomal neuraminidase : a unique member of the sialidase superfamily
E. Bonten
20001 citations
Semantic Scholar
Amino acid stress response genes promote L-asparaginase resistance in pediatric acute lymphoblastic leukemia
D. Ferguson, J. R. McCorkle, Kelly R. Barnett, E. Bonten +22 more
2022Blood Advances0 citations Open access
Semantic Scholar
MO006INFLAMMASOME ACTIVATOR NLRP3 HYPOMETHYLATION IS ASSOCIATED WITH GLUCOCORTICOID RESISTANCE IN PATIENTS WITH IDIOPATHIC NEPHROTIC SYNDROME
S. Granata, G. Stocco, M. Lucafò, E. Bonten +5 more
20200 citations
Semantic Scholar
bone marrow hematopoietic progenitor cells Functional amelioration of murine galactosialidosis by genetically modified
A. Allay, J. Cunningham, A. Nienhuis, Richard J Smeyne +5 more
20130 citations
Semantic Scholar
P5.66 The role of Neuraminidase 1 on skeletal muscle regeneration
Juliana de Carvalho Neves, V. R. Rizzato, Alan Fappi, T. S. Godoy +6 more
20110 citations
Semantic Scholar
35. NEU1 is a negative regulator of lysosomal exocytosis
A. d'Azzo, E. Bonten, D. van de Vlekkert, G. Yogalingam
20090 citations
Semantic Scholar
G.P.5 05 Skeletal muscle involvement in neuraminidase deficient mice
E. Zanoteli, Gouri Yogalingam, Hui-Min Hu, E. Bonten +1 more
20060 citations
Semantic Scholar
Susceptibility to asthma phenotype in human airway smooth muscle mannose receptor (huASM-MR) transgenic mice
W. Robinson, C. Michael, T. Overbeck, F. Kirkland +3 more
20040 citations
Semantic Scholar
Neuraminidase, Lysosomal (Sialidase)
A. d'Azzo, E. Bonten
20020 citations
Semantic Scholar

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