Bonten, Erik J.

Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.

Bonten, E., van der Spoel, A., Fornerod, M., Grosveld, G., & d'Azzo, A. (1996) · Genes & Development

Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and Myoclonus

Cited by 313

Neuraminidase 1 Is a Negative Regulator of Lysosomal Exocytosis

Yogalingam, G., Bonten, E. J., van de Vlekkert, D., Hu, H., Moshiach, S., Connell, S. A., & d'Azzo, A. (2008) · Developmental Cell

Cellular transport and secretionGlycosylation and Glycoproteins Research

Cited by 185

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

Paugh, S. W., Bonten, E. J., Savic, D., Ramsey, L. B., Thierfelder, W. E., Gurung, P., Malireddi, R. K. S., Actis, M., Mayasundari, A., Min, J., et al. (2015) · Nature Genetics

Acute Lymphoblastic Leukemia researchInflammasome and immune disorders

Cited by 164

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

Galjart, N. J., Morreau, H., Willemsen, R., Gillemans, N., Bonten, E. J., & d'Azzo, A. (1991) · Journal of Biological Chemistry

Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and Myoclonus

Cited by 158

Lysosomal multienzyme complex: pros and cons of working together

Bonten, E. J., Annunziata, I., & d’Azzo, A. (2014) · Cellular and Molecular Life Sciences

Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and Myoclonus

Cited by 117

Three-dimensional structure of the human protective protein: structure of the precursor form suggests a complex activation mechanism

Rudenko, G., Bonten, E., d'Azzo, A., & GJ Hol, W. (1995) · Structure

Enzyme Structure and FunctionLysosomal Storage Disorders Research

Cited by 97

Heterodimerization of the Sialidase NEU1 with the Chaperone Protective Protein/Cathepsin A Prevents Its Premature Oligomerization

Bonten, E. J., Campos, Y., Zaitsev, V., Nourse, A., Waddell, B., Lewis, W., Taylor, G., & d'Azzo, A. (2009) · Journal of Biological Chemistry

Lysosomal Storage Disorders ResearchGlycosylation and Glycoproteins Research

Cited by 87

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

Leimig, T., Mann, L., Martin, M. D. P., Bonten, E., Persons, D., Knowles, J., Allay, J. A., Cunningham, J., Nienhuis, A. W., Smeyne, R., et al. (2002) · Blood

Lysosomal Storage Disorders ResearchVirus-based gene therapy research

Cited by 75

Lysosomal Protective Protein/Cathepsin A

Bonten, E. J., Galjart, N. J., Willemsen, R., Usmany, M., Vlak, J. M., & d'Azzo, A. (1995) · Journal of Biological Chemistry

Protease and Inhibitor MechanismsCellular transport and secretion

Cited by 59

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

Bonten, E. J., Wang, D., Toy, J. N., Mann, L., Mignardot, A., Yogalingam, G., & d'Azzo, A. (2004) · The FASEB Journal

Lysosomal Storage Disorders ResearchViral Infectious Diseases and Gene Expression in Insects

Cited by 54

Lysosomal Neuraminidase

Bonten, E. J., & d'Azzo, A. (2000) · Journal of Biological Chemistry

Glycosylation and Glycoproteins ResearchLysosomal Storage Disorders Research

Cited by 51

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

Wang, D., Bonten, E. J., Yogalingam, G., Mann, L., & d’Azzo, A. (2005) · Molecular Genetics and Metabolism

Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and Myoclonus

Cited by 50

Preclinical Dose-Finding Study With a Liver-Tropic, Recombinant AAV-2/8 Vector in the Mouse Model of Galactosialidosis

Hu, H., Gomero, E., Bonten, E., Gray, J. T., Allay, J., Wu, Y., Wu, J., Calabrese, C., Nienhuis, A., & d'Azzo, A. (2012) · Molecular Therapy

Lysosomal Storage Disorders ResearchVirus-based gene therapy research

Cited by 37

The atomic model of the human protective protein/cathepsin A suggests a structural basis for galactosialidosis

Rudenko, G., Bonten, E., Hol, W. G. J., & d’Azzo, A. (1998) · Proceedings of the National Academy of Sciences

Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and Myoclonus

Cited by 36

Synthetic ZFTA fusions pinpoint disordered protein domain acquisition as a mechanism of brain tumorigenesis

Arabzade, A., Shirnekhi, H. K., Varadharajan, S., Ippagunta, S. M., Phillips, A. H., Laboe, N., Baggett, D. W., Wahiduzzaman, Jo, M., Zheng, T., et al. (2025) · Nature Cell Biology

Genomics and Chromatin DynamicsGlioma Diagnosis and Treatment

Cited by 7