J Q Trojanowski

Summary

Affiliation: University of Pennsylvania
Country: USA

Publications

  1. ncbi request reprint Enhanced neurofibrillary tangle formation, cerebral atrophy, and cognitive deficits induced by repetitive mild brain injury in a transgenic tauopathy mouse model
    Yasumasa Yoshiyama
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    J Neurotrauma 22:1134-41. 2005
  2. ncbi request reprint Novel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins
    Linda K Kwong
    Center for Neurodegenerative Disease Research and Institute on Aging, Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104 4283, USA
    Acta Neuropathol Commun 2:33. 2014
  3. ncbi request reprint Neuronal injury biomarkers and prognosis in ADNI subjects with normal cognition
    Jon B Toledo
    Department of Pathology and Laboratory Medicine, Institute on Aging, Center for Neurodegenerative Disease Research, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
    Acta Neuropathol Commun 2:26. 2014
  4. pmc Diagnostic effectiveness of quantitative [¹⁸F]flutemetamol PET imaging for detection of fibrillar amyloid β using cortical biopsy histopathology as the standard of truth in subjects with idiopathic normal pressure hydrocephalus
    Ville Leinonen
    Life Sciences, GE Healthcare, 101 Carnegie Center, Princeton, NJ 08540, USA
    Acta Neuropathol Commun 2:46. 2014
  5. ncbi request reprint Comparing metabolomic and pathologic biomarkers alone and in combination for discriminating Alzheimer's disease from normal cognitive aging
    Alison A Motsinger-Reif
    Department of Psychiatry and Behavioral Sciences, Duke University, Durham, NC, USA
    Acta Neuropathol Commun 1:28. 2013
  6. ncbi request reprint Expression of TMEM106B, the frontotemporal lobar degeneration-associated protein, in normal and diseased human brain
    Johanna I Busch
    Departments of Neurology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
    Acta Neuropathol Commun 1:36. 2013
  7. ncbi request reprint Clinical and multimodal biomarker correlates of ADNI neuropathological findings
    Jon B Toledo
    Department of Pathology and Laboratory Medicine, Institute on Aging, Center for Neurodegenerative Disease Research, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
    Acta Neuropathol Commun 1:65. 2013
  8. pmc The unfolded protein response is activated in disease-affected brain regions in progressive supranuclear palsy and Alzheimer's disease
    Lauren D Stutzbach
    Department of Pathology and Laboratory Medicine, 3630 Hamilton Walk, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    Acta Neuropathol Commun 1:31. 2013
  9. pmc Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U
    Edward B Lee
    Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA Translational Neuropathology Research Laboratory, 605B Stellar Chance Laboratories, 422 Curie Blvd, Philadelphia, PA 19104, USA Department of Pathology and Laboratory Medicine, Philadelphia, PA, USA
    Acta Neuropathol Commun 1:1-11. 2013
  10. pmc A model for improving the treatment and care of Alzheimer's disease patients through interdisciplinary research
    John Q Trojanowski
    Institute on Aging, University of Pennsylvania, Philadelphia, PA, USA
    Alzheimers Dement 8:564-73. 2012

Detail Information

Publications174 found, 100 shown here

  1. ncbi request reprint Enhanced neurofibrillary tangle formation, cerebral atrophy, and cognitive deficits induced by repetitive mild brain injury in a transgenic tauopathy mouse model
    Yasumasa Yoshiyama
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    J Neurotrauma 22:1134-41. 2005
    ..The reasons for the augmentation of tau pathologies in only one T44 tau Tg mouse subjected to mrTBI remain to be elucidated...
  2. ncbi request reprint Novel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins
    Linda K Kwong
    Center for Neurodegenerative Disease Research and Institute on Aging, Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104 4283, USA
    Acta Neuropathol Commun 2:33. 2014
    ..These data suggest that the novel MAbs reported here will be useful for patient-oriented research as well as for studies of animal and cell-based models of TDP-43 proteinopathies including ALS and FTLD-TDP. ..
  3. ncbi request reprint Neuronal injury biomarkers and prognosis in ADNI subjects with normal cognition
    Jon B Toledo
    Department of Pathology and Laboratory Medicine, Institute on Aging, Center for Neurodegenerative Disease Research, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
    Acta Neuropathol Commun 2:26. 2014
    ..Therefore, we analyzed data from 486 cognitively normal (CN) and 327 DAT subjects in the AD Neuroimaging Initiative (ADNI)-1/GO/2 cohorts...
  4. pmc Diagnostic effectiveness of quantitative [¹⁸F]flutemetamol PET imaging for detection of fibrillar amyloid β using cortical biopsy histopathology as the standard of truth in subjects with idiopathic normal pressure hydrocephalus
    Ville Leinonen
    Life Sciences, GE Healthcare, 101 Carnegie Center, Princeton, NJ 08540, USA
    Acta Neuropathol Commun 2:46. 2014
    ..prospective studies...
  5. ncbi request reprint Comparing metabolomic and pathologic biomarkers alone and in combination for discriminating Alzheimer's disease from normal cognitive aging
    Alison A Motsinger-Reif
    Department of Psychiatry and Behavioral Sciences, Duke University, Durham, NC, USA
    Acta Neuropathol Commun 1:28. 2013
    ..This would aid intervention trials designed to slow the progression of AD by increasing diagnostic certainty, and provide new pathophysiologic clues and potential drug targets...
  6. ncbi request reprint Expression of TMEM106B, the frontotemporal lobar degeneration-associated protein, in normal and diseased human brain
    Johanna I Busch
    Departments of Neurology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
    Acta Neuropathol Commun 1:36. 2013
    ..Indeed, TMEM106B expression levels, which correlate with TMEM106B genotype, may play a role in the pathogenesis of disease...
  7. ncbi request reprint Clinical and multimodal biomarker correlates of ADNI neuropathological findings
    Jon B Toledo
    Department of Pathology and Laboratory Medicine, Institute on Aging, Center for Neurodegenerative Disease Research, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
    Acta Neuropathol Commun 1:65. 2013
    ..Clinical data, neuropsychological measures, cerebrospinal fluid Aβ, total and phosphorylated tau and α-synuclein and MRI and FDG-PET scans...
  8. pmc The unfolded protein response is activated in disease-affected brain regions in progressive supranuclear palsy and Alzheimer's disease
    Lauren D Stutzbach
    Department of Pathology and Laboratory Medicine, 3630 Hamilton Walk, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    Acta Neuropathol Commun 1:31. 2013
    ..Here, we evaluate UPR PERK activation in the pons, medulla, midbrain, hippocampus, frontal cortex and cerebellum in subjects with PSP, AD, and in normal controls...
  9. pmc Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U
    Edward B Lee
    Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA Translational Neuropathology Research Laboratory, 605B Stellar Chance Laboratories, 422 Curie Blvd, Philadelphia, PA 19104, USA Department of Pathology and Laboratory Medicine, Philadelphia, PA, USA
    Acta Neuropathol Commun 1:1-11. 2013
    ..In this study, we report the clinical and pathologic features of three cases of aFTLD-U and two cases of late-onset BIBD with an emphasis on the anatomic distribution of FUS inclusions...
  10. pmc A model for improving the treatment and care of Alzheimer's disease patients through interdisciplinary research
    John Q Trojanowski
    Institute on Aging, University of Pennsylvania, Philadelphia, PA, USA
    Alzheimers Dement 8:564-73. 2012
    ..The "Ware-UPenn" program has been presented in this report as a useful prototype for partnerships between private philanthropy and academia in planning and developing programs to address a major national public health problem...
  11. pmc Phosphorylated tau/amyloid beta 1-42 ratio in ventricular cerebrospinal fluid reflects outcome in idiopathic normal pressure hydrocephalus
    Sunil Patel
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Fluids Barriers CNS 9:7. 2012
    ..abstract:..
  12. pmc Acetylated tau, a novel pathological signature in Alzheimer's disease and other tauopathies
    David J Irwin
    Centre for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Alzheimer s Disease Core Centre, Institute on Ageing, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6021, USA
    Brain 135:807-18. 2012
    ..Thus, inhibiting tau acetylation could be a disease-modifying target for drug discovery target in tauopathies...
  13. ncbi request reprint Proposed neuropathological criteria for the post mortem diagnosis of multiple system atrophy
    J Q Trojanowski
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Institute on Ageing of the University of Pennsylvania, Philadelphia, PA 19104 4283, USA
    Neuropathol Appl Neurobiol 33:615-20. 2007
    ..The deliberations and recommendations of the Working Group on Diagnostic Neuropathology Criteria for MSA are summarized in this report...
  14. ncbi request reprint Overview of protein aggregation in single, double, and triple neurodegenerative brain amyloidoses
    John Q Trojanowski
    Department of Pathology and Laboratory Medicine, Institute on Aging, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Neuromolecular Med 4:1-6. 2003
  15. ncbi request reprint "Fatal attractions" of proteins. A comprehensive hypothetical mechanism underlying Alzheimer's disease and other neurodegenerative disorders
    J Q Trojanowski
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, HUP, Maloney Building, 3rd Floor, Philadelphia, Pennsylvania 19104 4283, USA
    Ann N Y Acad Sci 924:62-7. 2000
    ....
  16. ncbi request reprint Living Longer and Paying the Price?
    John Q Trojanowski
    The Institute on Aging, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Sci Aging Knowledge Environ 2005:pe38. 2005
    ..Further, speakers presented their views on covering the costs of public and private programs for future generations of older adults...
  17. pmc Rous-Whipple Award Lecture. The Alzheimer's brain: finding out what's broken tells us how to fix it
    John Q Trojanowski
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Maloney 3, HUP, 3600 Spruce St, Philadelphia, PA 19104 4283, USA
    Am J Pathol 167:1183-8. 2005
  18. ncbi request reprint The art and science of anti-aging therapies
    John Q Trojanowski
    Institute on Aging and the Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Sci Aging Knowledge Environ 2005:pe11. 2005
    ..The program, which combined medical, surgical, and nonpharmacological approaches to healthy successful aging, gave attendees the opportunity to make sense of the issues at hand and to sort out safe treatments from perilous quick fixes...
  19. ncbi request reprint Parkinson's disease and related synucleinopathies are a new class of nervous system amyloidoses
    John Q Trojanowski
    Department of Pathology, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Neurotoxicology 23:457-60. 2002
    ..Continuing advances in this research direction should advance understanding of PD and accelerate discovery of more effective therapies for this and related synucleinopathies...
  20. ncbi request reprint Protein mis-folding emerges as a "drugable" target for discovery of novel therapies for neuropsychiatric diseases of aging
    John Q Trojanowski
    The Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Institute on Aging, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Am J Geriatr Psychiatry 12:134-5. 2004
  21. pmc Neuropathological verisimilitude in animal models of Alzheimer's disease: key to elucidating neurodegenerative pathways and identifying new targets for drug discovery
    John Q Trojanowski
    Department of Pathology and Laboratory Medicine, Division of Anatomical Pathology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Am J Pathol 160:409-11. 2002
  22. ncbi request reprint Meeting summary--cell biology of Parkinson's disease and related neurodegenerative disorders
    John Q Trojanowski
    Center for Neurodegenerative Disease Research, Institute on Aging and Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Sci Aging Knowledge Environ 2003:PE23. 2003
    ....
  23. ncbi request reprint Parkinson's disease and related alpha-synucleinopathies are brain amyloidoses
    John Q Trojanowski
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Ann N Y Acad Sci 991:107-10. 2003
    ..Thus, PD and related synucleinopathies are brain amyloidoses that may share similar mechanisms and targets for drug discovery...
  24. ncbi request reprint "Emerging Alzheimer's disease therapies: focusing on the future"
    John Q Trojanowski
    Department of Pathology and Laboratory Medicine, The Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia 19104 4283, USA
    Neurobiol Aging 23:985-90. 2002
    ..The symposium is summarized here followed by reviews from symposium speakers who describe potential anti-Abeta therapies some of which are in clinical trials...
  25. ncbi request reprint The role of tau in Alzheimer's disease
    John Q Trojanowski
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Maloney Building, 3rd Floor, HUP, Philadelphia, PA 19104, USA
    Med Clin North Am 86:615-27. 2002
    ....
  26. ncbi request reprint PENN neurodegenerative disease research - in the spirit of Benjamin Franklin
    John Q Trojanowski
    Institute on Aging, Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Neurosignals 16:5-10. 2008
    ..This research could enhance our chances of aging successfully in the continuing longevity revolution, and the essay here provides context and background on this research...
  27. pmc Novel CSF biomarkers for frontotemporal lobar degenerations
    W T Hu
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia 19104 4283, USA
    Neurology 75:2079-86. 2010
    ..To identify antemortem CSF diagnostic biomarkers that can potentially distinguish between the 2 main causes of frontotemporal lobar degeneration (FTLD), i.e., FTLD with TDP-43 pathology (FTLD-TDP) and FTLD with tau pathology (FTLD-tau)...
  28. pmc Update on the biomarker core of the Alzheimer's Disease Neuroimaging Initiative subjects
    John Q Trojanowski
    Department of Pathology and Laboratory Medicine, Institute on Aging, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Alzheimers Dement 6:230-8. 2010
    ..Further studies in ADNI will refine this model and render the biomarkers studied in ADNI more applicable to routine diagnosis and to clinical trials of disease modifying therapies...
  29. ncbi request reprint Transgenic models of tauopathies and synucleinopathies
    J Q Trojanowski
    The Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, The University of Pennsylvania School of Medicine, Philadelphia 19104 4283, USA
    Brain Pathol 9:733-9. 1999
    ....
  30. pmc Neocortical and hippocampal amyloid-β and tau measures associate with dementia in the oldest-old
    John L Robinson
    Center for Neurodegenerative Disease Research, Institute on Ageing, Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    Brain 134:3708-15. 2011
    ....
  31. pmc New directions for frontotemporal dementia drug discovery
    John Q Trojanowski
    Institute on Aging, Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Alzheimers Dement 4:89-93. 2008
    ..The recognition that almost all forms of FTD are due to TDP-43 proteinopathies and tauopathies creates new opportunities for FTD drug discovery targeting pathways of TDP-43 and tau-mediated neurodegeneration...
  32. pmc Design of comprehensive Alzheimer's disease centers to address unmet national needs
    John Q Trojanowski
    Institute on Aging, Philadelphia, PA, USA
    Alzheimers Dement 6:150-5. 2010
    ..The intent of this position paper is to stimulate thinking and foster the development of other or alternative models for a systematic approach to the study of dementia and movement disorders...
  33. ncbi request reprint Oxidative damage linked to neurodegeneration by selective alpha-synuclein nitration in synucleinopathy lesions
    B I Giasson
    Center for Neurodegenerative Disease Research and Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
    Science 290:985-9. 2000
    ..The selective and specific nitration of alpha-synuclein in these disorders provides evidence to directly link oxidative and nitrative damage to the onset and progression of neurodegenerative synucleinopathies...
  34. ncbi request reprint Radioiodinated styrylbenzenes and thioflavins as probes for amyloid aggregates
    Z P Zhuang
    Departments of Radiology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Med Chem 44:1905-14. 2001
    ....
  35. pmc Brain trauma induces massive hippocampal neuron death linked to a surge in beta-amyloid levels in mice overexpressing mutant amyloid precursor protein
    D H Smith
    Department of Neurosurgery, University of Pennsylvania, Philadelphia 19104 6316, USA
    Am J Pathol 153:1005-10. 1998
    ..01). These data suggest a mechanistic link between brain trauma and Abeta levels and the death of neurons...
  36. ncbi request reprint Absence of neurodegeneration and neural injury in the cerebral cortex in a sample of elderly patients with schizophrenia
    S E Arnold
    Department of Psychiatry, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Arch Gen Psychiatry 55:225-32. 1998
    ..However, we hypothesized that relatively small accumulations of age- or disease-related neurodegenerative lesions occurring in an otherwise abnormal brain could result in deterioration in schizophrenia...
  37. ncbi request reprint Attenuated neurodegenerative disease phenotype in tau transgenic mouse lacking neurofilaments
    T Ishihara
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 21:6026-35. 2001
    ....
  38. ncbi request reprint Glial cytoplasmic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble alpha-synuclein
    P H Tu
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA
    Ann Neurol 44:415-22. 1998
    ....
  39. ncbi request reprint Autosomal dominant dementia with widespread neurofibrillary tangles
    L A Reed
    Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City 52242, USA
    Ann Neurol 42:564-72. 1997
    ....
  40. ncbi request reprint Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration
    S X Xie
    Department of Biostatistics and Epidemiology, University of Pennsylvania School of Medicine Philadelphia, PA, USA
    J Neurol Neurosurg Psychiatry 79:126-9. 2008
    ..To examine the clinical and pathological factors associated with survival in autopsy-confirmed frontotemporal lobar degeneration (FTLD)...
  41. pmc CSF amyloid {beta} 1-42 predicts cognitive decline in Parkinson disease
    A Siderowf
    Department of Neurology, 330 South 9th Street, Second Floor, Philadelphia, PA 19107, USA
    Neurology 75:1055-61. 2010
    ..Cognitive decline associated with Parkinson disease (PD) is common and highly disabling. Biomarkers that help identify patients at risk for cognitive decline would be useful additions to the clinical management of the disease...
  42. ncbi request reprint Isomerization of (Z,Z) to (E,E)1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene in strong base: probes for amyloid plaques in the brain
    C W Lee
    Department of Radiology, University of Pennsylvania, 3700 Market Street, Room 305, Philadelphia, Pennsylvania 19104, USA
    J Med Chem 44:2270-5. 2001
    ..Such information could be exploited to develop new ligands for detecting amyloid plaques in AD...
  43. ncbi request reprint Tau isoform profile and phosphorylation state in dementia pugilistica recapitulate Alzheimer's disease
    M L Schmidt
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104-4283, USA
    Acta Neuropathol 101:518-24. 2001
    ....
  44. ncbi request reprint Vitamin E reduces amyloidosis and improves cognitive function in Tg2576 mice following repetitive concussive brain injury
    Valeria Conte
    Head Injury Center, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    J Neurochem 90:758-64. 2004
    ..This study suggests that the exacerbation of brain oxidative stress following RCBI plays a mechanistic role in accelerating Alphabeta accumulation and behavioral impairments in the Tg2576 mice...
  45. ncbi request reprint Repetitive mild brain trauma accelerates Abeta deposition, lipid peroxidation, and cognitive impairment in a transgenic mouse model of Alzheimer amyloidosis
    Kunihiro Uryu
    Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4283, USA
    J Neurosci 22:446-54. 2002
    ....
  46. ncbi request reprint Retarded axonal transport of R406W mutant tau in transgenic mice with a neurodegenerative tauopathy
    Bin Zhang
    The Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, and Institute on Aging, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4283, USA
    J Neurosci 24:4657-67. 2004
    ....
  47. ncbi request reprint Cognitive and motor assessment in autopsy-proven corticobasal degeneration
    R Murray
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Neurology 68:1274-83. 2007
    ..To investigate the clinical features of autopsy-proven corticobasal degeneration (CBD)...
  48. ncbi request reprint Frontotemporal dementia progresses to death faster than Alzheimer disease
    E D Roberson
    Memory and Aging Center, Department of Neurology, University of California, San Francisco, CA, USA
    Neurology 65:719-25. 2005
    ..Frontotemporal lobar degeneration (FTLD) is a common cause of non-Alzheimer dementia, but its natural history and the factors related to mortality in affected patients are not well understood...
  49. ncbi request reprint A panel of epitope-specific antibodies detects protein domains distributed throughout human alpha-synuclein in Lewy bodies of Parkinson's disease
    B I Giasson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19104 4283, USA
    J Neurosci Res 59:528-33. 2000
    ....
  50. ncbi request reprint Prominent perikaryal expression of alpha- and beta-synuclein in neurons of dorsal root ganglion and in medullary neurons
    B I Giasson
    Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Exp Neurol 172:354-62. 2001
    ....
  51. pmc Longitudinal decline in autopsy-defined frontotemporal lobar degeneration
    M Grossman
    Department of Neurology, University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104 4283, USA
    Neurology 70:2036-45. 2008
    ..The natural history of patients with pathologically proven frontotemporal lobar degeneration (FTLD) is important from clinical and biologic perspectives, but is not well documented quantitatively...
  52. ncbi request reprint Quantitative neurohistological features of frontotemporal degeneration
    S E Arnold
    Department of Psychiatry, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Neurobiol Aging 21:913-9. 2000
    ..The molecular neurodegenerative mechanisms that lead to FTD remain to be elucidated...
  53. ncbi request reprint Spinal cord neurofibrillary pathology in Alzheimer disease and Guam Parkinsonism-dementia complex
    M L Schmidt
    Center for Neurodegenerative Disease Research, University of Pennsylvania, Philadelphia 19104-4283, USA
    J Neuropathol Exp Neurol 60:1075-86. 2001
    ..Western blot analysis confirmed this by revealing that sarcosyl insoluble tau in spinal cord extracts from patients with NFTs exhibited the presence of all 6 tau isoforms similar to that from AD and ALS/PDC cortical gray matter...
  54. ncbi request reprint Chaperone suppression of alpha-synuclein toxicity in a Drosophila model for Parkinson's disease
    Pavan K Auluck
    Department of Neuroscience, University of Pennsylvania and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Science 295:865-8. 2002
    ..Furthermore, Lewy bodies in human postmortem tissue immunostained for molecular chaperones, also suggesting that chaperones may play a role in Parkinson's disease progression...
  55. ncbi request reprint Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
    Manuela Neumann
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Science 314:130-3. 2006
    ..TDP-43 represents the common pathologic substrate linking these neurodegenerative disorders...
  56. pmc BACE overexpression alters the subcellular processing of APP and inhibits Abeta deposition in vivo
    Edward B Lee
    The Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine
    J Cell Biol 168:291-302. 2005
    ....
  57. ncbi request reprint Initiation and synergistic fibrillization of tau and alpha-synuclein
    Benoit I Giasson
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine
    Science 300:636-40. 2003
    ..This suggests that interactions between alpha-syn and tau can promote their fibrillization and drive the formation of pathological inclusions in human neurodegenerative diseases...
  58. pmc Neurodegeneration with brain iron accumulation, type 1 is characterized by alpha-, beta-, and gamma-synuclein neuropathology
    J E Galvin
    Department of Neurology, Medical College of Pennsylvania Hahnemann University the Center for Neurodegenerative Disease Research, and the Department of Neurology, The University of Pennsylvania, Philadelphia, Pennsylvania, USA
    Am J Pathol 157:361-8. 2000
    ..These findings expand the concept of neurodegenerative synucleinopathies by implicating alphaS, betaS, and gammaS in the pathogenesis of NBIA 1...
  59. ncbi request reprint Abnormal tau phosphorylation at Ser396 in Alzheimer's disease recapitulates development and contributes to reduced microtubule binding
    G T Bramblett
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 4283
    Neuron 10:1089-99. 1993
    ..Thus, phosphorylation of Ser396 may destabilize MTs in AD, resulting in the degeneration of affected cells...
  60. pmc Age-dependent induction of congophilic neurofibrillary tau inclusions in tau transgenic mice
    T Ishihara
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Am J Pathol 158:555-62. 2001
    ....
  61. pmc Axon pathology in Parkinson's disease and Lewy body dementia hippocampus contains alpha-, beta-, and gamma-synuclein
    J E Galvin
    Department of Neurology, Medical College of Pennsylvania, Hahnemann University, 245 North 15th Street, Philadelphia, PA 19129, USA
    Proc Natl Acad Sci U S A 96:13450-5. 1999
    ..Our findings broaden the concept of neurodegenerative "synucleinopathies" by implicating betaS and gammaS, in addition to alphaS, in the onset/progression of PD and DLB...
  62. ncbi request reprint Loss of brain tau defines novel sporadic and familial tauopathies with frontotemporal dementia
    V Zhukareva
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, USA
    Ann Neurol 49:165-75. 2001
    ..The presence of tau mRNA in these brains suggests that the level of tau protein may be controlled posttranscriptionally, at the level of either translation or mRNA stability...
  63. ncbi request reprint AMY plaques in familial AD: comparison with sporadic Alzheimer's disease
    C F Lippa
    Department of Neurology, MCP Hahnemann University, Philadelphia, PA 19102, USA
    Neurology 54:100-4. 2000
    ..To assess AMY expression in familial AD (FAD)...
  64. ncbi request reprint Alpha-synuclein cortical Lewy bodies correlate with dementia in Parkinson's disease
    H I Hurtig
    Department of Neurology, University of Pennsylvania, PA, USA
    Neurology 54:1916-21. 2000
    ..Although Alzheimer's pathology has been a frequent finding, recent advances in immunostaining of alpha-synuclein have suggested the possible importance of cortical Lewy bodies (CLBs) in the brains of demented patients with PD...
  65. ncbi request reprint Cortical biochemistry in MCI and Alzheimer disease: lack of correlation with clinical diagnosis
    M S Forman
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 422 Curie Blvd, 605B Stellar Chance Building, Philadelphia, PA 19104, USA
    Neurology 68:757-63. 2007
    ..Mild cognitive impairment, hypothesized to be prodromal Alzheimer disease (AD), shows abundant senile plaques and neurofibrillary tangles, but its biochemical correlates remain undefined...
  66. ncbi request reprint Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein
    Benoit I Giasson
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, 3600 Spruce Street, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Neuron 34:521-33. 2002
    ..These mice demonstrate that A53T alpha-synuclein leads to the formation of toxic filamentous alpha-synuclein neuronal inclusions that cause neurodegeneration...
  67. ncbi request reprint Early vitamin E supplementation in young but not aged mice reduces Abeta levels and amyloid deposition in a transgenic model of Alzheimer's disease
    Syuan Sung
    Center for Experimental Therapeutics, Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    FASEB J 18:323-5. 2004
    ..These results support the hypothesis that oxidative stress is an important early event in AD pathogenesis, and antioxidant therapy may be beneficial only if given at this stage of the disease process...
  68. ncbi request reprint Cerebrospinal fluid profile in frontotemporal dementia and Alzheimer's disease
    Murray Grossman
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Ann Neurol 57:721-9. 2005
    ..We conclude that CSF tau levels are significantly reduced in many patients with FTD...
  69. ncbi request reprint Age-dependent synuclein pathology following traumatic brain injury in mice
    K Uryu
    The Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Exp Neurol 184:214-24. 2003
    ..This model of age-dependent TBI-induced transient alterations in alpha-Syn provides an opportunity to examine possible links between TBI and mechanisms of disease in synucleinopathies...
  70. pmc Multimodal predictors for Alzheimer disease in nonfluent primary progressive aphasia
    W T Hu
    Department of Neurology, University of Pennsylvania School of Medicine, 3400 Spruce Street, Philadelphia, PA 19106, USA
    Neurology 75:595-602. 2010
    ..We sought to determine the value of clinical characterization, neuropsychological analysis, and MRI atrophy in predicting pathology of LPA and PNFA...
  71. ncbi request reprint Early fetal acquisition of the chromaffin and neuronal immunophenotype by human adrenal medullary cells. An immunohistological study using monoclonal antibodies to chromogranin A, synaptophysin, tyrosine hydroxylase, and neuronal cytoskeletal proteins
    W M Molenaar
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104
    Exp Neurol 108:1-9. 1990
    ..They suggest that "large" and "small" cells are progenitors of the chromaffin and the ganglion cells, respectively, of the mature adrenal medulla...
  72. ncbi request reprint Frontotemporal dementia: report of a familial case
    M Shafiq
    Department of Neurology, MCP-Hahnemann University, Philadelphia, PA, USA
    Neurology 56:S31-4. 2001
    ..Tau-positive glial cells were also present. The authors suggest that the abnormal tau aggregates are related to the symptoms experienced by affected members of this family...
  73. ncbi request reprint Glial fibrillary acidic protein-immunoreactive astrocytosis in elderly patients with schizophrenia and dementia
    S E Arnold
    Department of Psychiatry, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Acta Neuropathol 91:269-77. 1996
    ..In the absence of any diagnostic neuropathological findings in this subgroup, the implications of these observations for the pathogenesis of schizophrenia remain to be determined...
  74. ncbi request reprint Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis
    Felix Geser
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Alzheimer s Disease Core Center, Institute on Aging, Philadelphia, USA
    Arch Neurol 65:636-41. 2008
    ....
  75. ncbi request reprint Impaired glutamate transport in a mouse model of tau pathology in astrocytes
    Deepa V Dabir
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 26:644-54. 2006
    ..Thus, these Tg mice recapitulate features of astrocytic pathology observed in tauopathies and implicate a role for altered astrocyte function in the pathogenesis of these disorders...
  76. ncbi request reprint Synapse loss and microglial activation precede tangles in a P301S tauopathy mouse model
    Yasumasa Yoshiyama
    The Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Neuron 53:337-51. 2007
    ..Thus, hippocampal synaptic pathology and microgliosis may be the earliest manifestations of neurodegenerative tauopathies, and abrogation of tau-induced microglial activation could retard progression of these disorders...
  77. ncbi request reprint Mutant and wild type human alpha-synucleins assemble into elongated filaments with distinct morphologies in vitro
    B I Giasson
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 274:7619-22. 1999
    ....
  78. pmc Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease
    N J Cairns
    Center for Neurodegenerative Disease Research, Department University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Neurology 63:1376-84. 2004
    ..Recently described neuronal intermediate filament inclusion disease (NIFID) shows considerable clinical heterogeneity...
  79. ncbi request reprint Transgenic mouse models of tauopathies: prospects for animal models of Pick's disease
    V M Lee
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, The University of Pennsylvania School of Medicine, Philadelphia, USA
    Neurology 56:S26-30. 2001
    ....
  80. ncbi request reprint Frontotemporal dementia with novel tau pathology and a Glu342Val tau mutation
    C F Lippa
    Department of Neurology, Medical College of Pennsylvania Hahnemann University, Philadelphia 19129, USA
    Ann Neurol 48:850-8. 2000
    ....
  81. pmc The fluorescent Congo red derivative, (trans, trans)-1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene (BSB), labels diverse beta-pleated sheet structures in postmortem human neurodegenerative disease brains
    M L Schmidt
    Department of Pathology, University of Pennsylvania, Philadelphia 19104-4283, USA
    Am J Pathol 159:937-43. 2001
    ....
  82. ncbi request reprint Alpha-synuclein in familial Alzheimer disease: epitope mapping parallels dementia with Lewy bodies and Parkinson disease
    C F Lippa
    Department of Neurology, Medical College of Pennsylvania Hahnemann University, 3300 Henry Ave, Philadelphia, PA 19129, USA
    Arch Neurol 58:1817-20. 2001
    ..However, epitope mapping for alpha-synuclein is distinctive in different neurodegenerative diseases. The reasons for this are poorly understood but may reflect fundamental differences in disease mechanisms...
  83. ncbi request reprint Human fetal hippocampal development: II. The neuronal cytoskeleton
    S E Arnold
    Department of Psychiatry and Neurology, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Comp Neurol 367:293-307. 1996
    ..These observations have implications for understanding the role of the neuronal cytoskeleton in the developing, mature, and diseased CNS...
  84. pmc Frontotemporal dementia: clinicopathological correlations
    Mark S Forman
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Ann Neurol 59:952-62. 2006
    ..This study assessed whether specific clinical features predict the underlying pathology...
  85. pmc Nitration of tau protein is linked to neurodegeneration in tauopathies
    Takashi Horiguchi
    Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, Institute on Aging, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Am J Pathol 163:1021-31. 2003
    ..Taken together, these findings imply that nitrative injury is directly linked to the formation of filamentous tau inclusions...
  86. pmc Alzheimer's disease neuroimaging initiative: a one-year follow up study using tensor-based morphometry correlating degenerative rates, biomarkers and cognition
    Alex D Leow
    Laboratory of Neuro Imaging, Department of Neurology, UCLA School of Medicine, Los Angeles, CA 90095 1769, USA
    Neuroimage 45:645-55. 2009
    ....
  87. pmc Clinical and pathological continuum of multisystem TDP-43 proteinopathies
    Felix Geser
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Arch Neurol 66:180-9. 2009
    ....
  88. pmc Overexpression of the human NFM subunit in transgenic mice modifies the level of endogenous NFL and the phosphorylation state of NFH subunits
    P H Tu
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 4283, USA
    J Cell Biol 129:1629-40. 1995
    ..NFM and NFH proteins may assume similar functions in regulation of NF packing density in vivo...
  89. ncbi request reprint Mild head injury increasing the brain's vulnerability to a second concussive impact
    H L Laurer
    The Head Injury Center, Department of Neurosurgery, University of Pennsylvania School of Medicine, Philadelphia 19104-6316, USA
    J Neurosurg 95:859-70. 2001
    ..CONCLUSIONS: On the basis of their results, the authors suggest that the brain has an increased vulnerability to a second traumatic insult for at least 24 hours following an initial episode of mild brain trauma...
  90. ncbi request reprint RNA sequestration to pathological lesions of neurodegenerative diseases
    S D Ginsberg
    Center for Neurodegenerative Disease Research and Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104 4283, USA
    Acta Neuropathol 96:487-94. 1998
    ..These observations demonstrate the selective localization of RNA species to distinct pathological lesions of neurodegenerative disease brains...
  91. ncbi request reprint Selective degeneration fo Purkinje cells with Lewy body-like inclusions in aged NFHLACZ transgenic mice
    P H Tu
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Neurosci 17:1064-74. 1997
    ....
  92. pmc Corticobasal syndrome and primary progressive aphasia as manifestations of LRRK2 gene mutations
    A S Chen-Plotkin
    Center for Neurodegenerative Disease Research, Institute on Aging and Department of Pathology and Laboratory Medicine, University of Pennsylvania Health System, Philadelphia, PA 19104, USA
    Neurology 70:521-7. 2008
    ..Despite pleomorphic pathology, LRRK2 mutations are believed to manifest clinically as typical Parkinson disease (PD). However, most genetic screens have been limited to PD clinic populations...
  93. ncbi request reprint A review and rationale for the use of genetically engineered animals in the study of traumatic brain injury
    L Longhi
    Department of Neurosurgery, University of Pennsylvania and Veterans Administration Medical Center, Philadelphia, Pennsylvania 19104, USA
    J Cereb Blood Flow Metab 21:1241-58. 2001
    ....
  94. pmc TDP-43 pathology occurs infrequently in multiple system atrophy
    F Geser
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
    Neuropathol Appl Neurobiol 37:358-65. 2011
    ..Consequently, we examined MSA for evidence of TDP-43 or FUS pathology utilizing immunohistochemical studies in autopsy material from 29 MSA patients...
  95. ncbi request reprint Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease
    G M McKhann
    Department of Neurology, Zanvyl Krieger Mind Brain Institute, Johns Hopkins University School of Medicine, 338 Krieger Hall, 3400 N Charles St, Baltimore, MD 21218 2685, USA
    Arch Neurol 58:1803-9. 2001
    ..In addition, recommendations for the neuropathological criteria of FTD were reviewed, relative to classical neuropathology and modern molecular biology...
  96. ncbi request reprint Enhanced brain levels of 8,12-iso-iPF2alpha-VI differentiate AD from frontotemporal dementia
    Y Yao
    Center for Experimental Therapeutics, Department of Pharmacology, University of Pennsylvania, School of Medicine, Philadelphia 19104, USA
    Neurology 61:475-8. 2003
    ..To quantify the isoprostane 8,12-iso-iPF2alpha-VI in brain tissues obtained from patients with AD, patients with frontotemporal dementia (FTD), and controls...
  97. pmc Second consensus statement on the diagnosis of multiple system atrophy
    S Gilman
    Department of Neurology, University of Michigan, 300 N Ingalls St, 3D15, Ann Arbor, MI 48109 5489, USA
    Neurology 71:670-6. 2008
    ..Since then, clinical, laboratory, neuropathologic, and imaging studies have advanced the field, requiring a fresh evaluation of diagnostic criteria. We held a second consensus conference in 2007 and present the results here...
  98. pmc Lewy bodies contain altered alpha-synuclein in brains of many familial Alzheimer's disease patients with mutations in presenilin and amyloid precursor protein genes
    C F Lippa
    Department of Neurology, Allegheny University of the Health Sciences MCP Division, Philadelphia, Pennsylvania 19129, USA
    Am J Pathol 153:1365-70. 1998
    ..These studies suggest that insoluble alpha-synuclein aggregates into filaments that form LBs in many FAD patients, and we speculate that these inclusions may compromise the function and/or viability of affected neurons in the FAD brain...
  99. ncbi request reprint PP2A mRNA expression is quantitatively decreased in Alzheimer's disease hippocampus
    V Vogelsberg-Ragaglia
    Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Exp Neurol 168:402-12. 2001
    ..This could result in decreased protein expression and phosphatase activity, leading to the hyperphosphorylation of tau and the formation of NFTs, as well as neuron degeneration in AD...
  100. pmc Amyotrophic lateral sclerosis-plus syndrome with TAR DNA-binding protein-43 pathology
    Leo F McCluskey
    Department of Neurology, University of Pennsylvania School of Medicine, 330 S 9th St, Philadelphia, PA 19107, USA
    Arch Neurol 66:121-4. 2009
    ....
  101. pmc Long-term accumulation of amyloid-beta, beta-secretase, presenilin-1, and caspase-3 in damaged axons following brain trauma
    Xiao Han Chen
    Department of Neurosurgery, University of Pennsylvania, 105C Hayden Hall, 3320 SmithWalk, Philadelphia, PA 19104 6316, USA
    Am J Pathol 165:357-71. 2004
    ..The abnormal concentration of these factors may lead to APP proteolysis and Abeta formation within the axonal membrane compartment...