P C Wong

Summary

Affiliation: Johns Hopkins University
Country: USA

Publications

  1. ncbi The genetic and molecular mechanisms of motor neuron disease
    P C Wong
    Department of Pathology, Division of Neuropathology, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, Maryland 21205 2196, USA
    Curr Opin Neurobiol 8:791-9. 1998
  2. ncbi Presenilin 1 is required for Notch1 and DII1 expression in the paraxial mesoderm
    P C Wong
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Nature 387:288-92. 1997
  3. ncbi The value of transgenic models for the study of neurodegenerative diseases
    D L Price
    Division of Neuropathology, Johns Hopkins University School of Medicine, 558 Ross Research Building, 720 Rutland Avenue, Baltimore, Maryland 21205 2196, USA
    Ann N Y Acad Sci 920:179-91. 2000
  4. ncbi Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues
    M Watanabe
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA
    Neurobiol Dis 8:933-41. 2001
  5. ncbi Inherited neurodegenerative diseases and transgenic models
    D L Price
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205 2196, USA
    Brain Pathol 6:467-80. 1996
  6. ncbi An Alzheimer's disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos
    J A Davis
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Neuron 20:603-9. 1998
  7. ncbi Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics
    P C Wong
    Department of Pathology The Johns Hopkins University School of Medicine, Baltimore, MD 21205-2196, USA
    J Mol Neurosci 17:233-57. 2001
  8. ncbi Hyper-expression of human apolipoprotein E4 in astroglia and neurons does not enhance amyloid deposition in transgenic mice
    C Lesuisse
    Department of Pathology, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross Building Room 558, Baltimore, MD 21205, USA
    Hum Mol Genet 10:2525-37. 2001
  9. pmc Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase
    P C Wong
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Proc Natl Acad Sci U S A 97:2886-91. 2000
  10. ncbi Transgenic mouse models of Alzheimer's disease and amyotrophic lateral sclerosis
    D R Borchelt
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Brain Pathol 8:735-57. 1998

Collaborators

Detail Information

Publications20

  1. ncbi The genetic and molecular mechanisms of motor neuron disease
    P C Wong
    Department of Pathology, Division of Neuropathology, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, Maryland 21205 2196, USA
    Curr Opin Neurobiol 8:791-9. 1998
    ..Of particular interest is recent work on the pathogenic mechanisms underlying these diseases, especially studies in in vitro model systems and in transgenic and gene-targeted mice...
  2. ncbi Presenilin 1 is required for Notch1 and DII1 expression in the paraxial mesoderm
    P C Wong
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Nature 387:288-92. 1997
    ..Hence, PS1 is required for the spatiotemporal expression of Notch1 and Dll1, which are essential for somite segmentation and maintenance of somite borders...
  3. ncbi The value of transgenic models for the study of neurodegenerative diseases
    D L Price
    Division of Neuropathology, Johns Hopkins University School of Medicine, 558 Ross Research Building, 720 Rutland Avenue, Baltimore, Maryland 21205 2196, USA
    Ann N Y Acad Sci 920:179-91. 2000
    ..It is hoped such study will result in novel treatments for testing in transgenic models that can then be translated into new treatments for human neurodegenerative diseases...
  4. ncbi Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues
    M Watanabe
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA
    Neurobiol Dis 8:933-41. 2001
    ..Overall, these observations suggest that inclusions might be sequestered into ubiquitin-proteasome pathway and some chaperone proteins such as Hsc70 may be involved in formation and/or degradation of these inclusions...
  5. ncbi Inherited neurodegenerative diseases and transgenic models
    D L Price
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205 2196, USA
    Brain Pathol 6:467-80. 1996
    ..Investigations of these models have begun to provide new insights into the roles of disease-specific mutant proteins and the pathogenic mechanisms of disease as well as opportunities to test therapeutic interventions...
  6. ncbi An Alzheimer's disease-linked PS1 variant rescues the developmental abnormalities of PS1-deficient embryos
    J A Davis
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Neuron 20:603-9. 1998
    ..Collectively, these data lead us to conclude that mutant PS1 causes AD not by loss of normal PS1 function but by influencing amyloid precursor protein (APP) processing in a manner that elevates Abeta1-42/43 production...
  7. ncbi Genetically engineered models relevant to neurodegenerative disorders: their value for understanding disease mechanisms and designing/testing experimental therapeutics
    P C Wong
    Department of Pathology The Johns Hopkins University School of Medicine, Baltimore, MD 21205-2196, USA
    J Mol Neurosci 17:233-57. 2001
    ..Recent works on clarifying the selective vulnerability of neurons and pathogenic mechanisms using genetically engineered mouse models of familial forms of Alzheimer's disease and motor neuron disease will be reviewed...
  8. ncbi Hyper-expression of human apolipoprotein E4 in astroglia and neurons does not enhance amyloid deposition in transgenic mice
    C Lesuisse
    Department of Pathology, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross Building Room 558, Baltimore, MD 21205, USA
    Hum Mol Genet 10:2525-37. 2001
    ..We suggest that these data are best explained by proposing that the mechanisms by which Apo E influences A beta deposition involves an aspect of its normal function that is not augmented by hyper-expression...
  9. pmc Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase
    P C Wong
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Proc Natl Acad Sci U S A 97:2886-91. 2000
    ..These results demonstrate the essential role of any mammalian copper chaperone and have important implications for the development of novel therapeutic strategies in familial amyotrophic lateral sclerosis...
  10. ncbi Transgenic mouse models of Alzheimer's disease and amyotrophic lateral sclerosis
    D R Borchelt
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Brain Pathol 8:735-57. 1998
    ....
  11. ncbi An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
    P C Wong
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Neuron 14:1105-16. 1995
    ....
  12. ncbi The copper chaperone CCS is abundant in neurons and astrocytes in human and rodent brain
    J D Rothstein
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    J Neurochem 72:422-9. 1999
    ..That both SOD1 and CCS are present, together, in cells that degenerate in ALS also emphasizes the potential role of CCS in mutant SOD1-mediated toxicity...
  13. pmc Alteration of BACE1-dependent NRG1/ErbB4 signaling and schizophrenia-like phenotypes in BACE1-null mice
    A V Savonenko
    Departments of Pathology, Neurology, and Neuroscience, Johns Hopkins University School of Medicine, 558 Ross Research Building, 720 Rutland Avenue, Baltimore, MD 21205, USA
    Proc Natl Acad Sci U S A 105:5585-90. 2008
    ..Collectively, our findings suggest that alterations in BACE1-dependent NRG1/ErbB4 signaling may participate in the pathogenesis of schizophrenia and related psychiatric disorders...
  14. pmc Overexpression of human copper, zinc-superoxide dismutase (SOD1) prevents postischemic injury
    P Wang
    Molecular and Cellular Biophysics Laboratories, Department of Medicine, Division of Cardiology and the Electron Paramagnetic Resonance Center, The Johns Hopkins University School of Medicine, Johns Hopkins Bayview Medical Center, Baltimore, MD 21224, USA
    Proc Natl Acad Sci U S A 95:4556-60. 1998
    ..Thus, increasing intracellular SOD1 expression may be a highly effective approach to decrease the cellular injury that occurs following reperfusion of ischemic tissues...
  15. ncbi Stroke outcome in double-mutant antioxidant transgenic mice
    K Sampei
    Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21287 4961, USA
    Stroke 31:2685-91. 2000
    ....
  16. ncbi BACE1 is the major beta-secretase for generation of Abeta peptides by neurons
    H Cai
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Nat Neurosci 4:233-4. 2001
  17. pmc Increasing neurofilament subunit NF-M expression reduces axonal NF-H, inhibits radial growth, and results in neurofilamentous accumulation in motor neurons
    P C Wong
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Cell Biol 130:1413-22. 1995
    ..Increase in NF-M did not result in an overt phenotype or neuronal loss, although filamentous swellings in perikarya and proximal axons of motor neurons were frequently found...
  18. pmc Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
    S Couillard-Despres
    Centre for Research in Neuroscience, McGill University, Montreal General Hospital Research Institute, Montreal H3G 1A4 Canada
    Proc Natl Acad Sci U S A 95:9626-30. 1998
    ..Although massive neurodegeneration occurred in 1-yr-old mice expressing SOD1(G37R) alone, spinal root axons and motor neurons were remarkably spared in doubly SOD1(G37R);NF-H-transgenic littermates...
  19. ncbi Effects of PS1 deficiency on membrane protein trafficking in neurons
    S Naruse
    Department of Pharmacological and Physiological Sciences, The University of Chicago, Illinois 60637, USA
    Neuron 21:1213-21. 1998
    ..We conclude that PS1 plays an essential role in modulating trafficking and metabolism of a selected set of membrane and secretory proteins in neurons...
  20. ncbi Pathogenesis of two axonopathies does not require axonal neurofilaments
    J Eyer
    INSERM CJF 97 08 and University of Angers, CHU, France
    Nature 391:584-7. 1998
    ..By specifically excluding a necessary role for axonal neurofilaments, our observations redefine the components of the pathogenic pathway leading to axon disruption in these two degenerative diseases...