Erik J Bonten

Summary

Affiliation: St. Jude Children's Research Hospital
Country: USA

Publications

  1. pmc Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization
    Erik J Bonten
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    J Biol Chem 284:28430-41. 2009
  2. pmc Neuraminidase 1 is a negative regulator of lysosomal exocytosis
    Gouri Yogalingam
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, TN 38105 2794, USA
    Dev Cell 15:74-86. 2008
  3. pmc Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue
    Edmar Zanoteli
    Department of Genetics, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Biochim Biophys Acta 1802:659-72. 2010
  4. ncbi request reprint Short-term, high dose enzyme replacement therapy in sialidosis mice
    Dongning Wang
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, 332 N Lauderdale, Memphis, TN 38105 2794, USA
    Mol Genet Metab 85:181-9. 2005
  5. pmc Lysosomal multienzyme complex: pros and cons of working together
    Erik J Bonten
    Department of Pharmaceutical Sciences, St Jude Children s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105 2794, USA
    Cell Mol Life Sci 71:2017-32. 2014
  6. pmc Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis
    Erik J Bonten
    Department of Genetics, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Biochim Biophys Acta 1832:1784-92. 2013
  7. ncbi request reprint Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis
    Erik J Bonten
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    FASEB J 18:971-3. 2004

Collaborators

Detail Information

Publications7

  1. pmc Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization
    Erik J Bonten
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    J Biol Chem 284:28430-41. 2009
    ..The proposed mechanism of interaction between NEU1 and its accessory protein PPCA provides a rationale for the secondary deficiency of NEU1 in galactosialidosis...
  2. pmc Neuraminidase 1 is a negative regulator of lysosomal exocytosis
    Gouri Yogalingam
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, TN 38105 2794, USA
    Dev Cell 15:74-86. 2008
    ..Our findings uncover an unexpected mechanism influencing lysosomal exocytosis and argue that exacerbations of this process form the basis for certain genetic diseases...
  3. pmc Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue
    Edmar Zanoteli
    Department of Genetics, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Biochim Biophys Acta 1802:659-72. 2010
    ..More broadly, these findings point to a potential role of NEU1 in cell proliferation and extracellular matrix remodeling...
  4. ncbi request reprint Short-term, high dose enzyme replacement therapy in sialidosis mice
    Dongning Wang
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, 332 N Lauderdale, Memphis, TN 38105 2794, USA
    Mol Genet Metab 85:181-9. 2005
    ..However, beyond 2 weeks of treatment, ERT mice developed a severe immune response towards the exogenous Neu1 enzyme. These results may have important implications for ERT in sialidosis patients...
  5. pmc Lysosomal multienzyme complex: pros and cons of working together
    Erik J Bonten
    Department of Pharmaceutical Sciences, St Jude Children s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105 2794, USA
    Cell Mol Life Sci 71:2017-32. 2014
    ..These findings have broadened our perspective on their functions and may pave the way for the development of new therapies for these lysosomal storage disorders. ..
  6. pmc Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis
    Erik J Bonten
    Department of Genetics, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Biochim Biophys Acta 1832:1784-92. 2013
    ..This resulted in clear amelioration of the disease phenotype. These results suggest that at least some of the NEU1 mutations associated with type I sialidosis may respond to PPCA-chaperone-mediated gene therapy. ..
  7. ncbi request reprint Targeting macrophages with baculovirus-produced lysosomal enzymes: implications for enzyme replacement therapy of the glycoprotein storage disorder galactosialidosis
    Erik J Bonten
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    FASEB J 18:971-3. 2004
    ..Our results suggest that ERT with baculovirus-expressed enzymes might be an effective treatment for nonneuropathic patients with galactosialidosis and possibly for others with LSDs that primarily involve the RE system...