DENNIS WILLIAM DICKSON

Summary

Affiliation: Mayo Clinic
Country: USA

Publications

  1. pmc Misfolded, protease-resistant proteins in animal models and human neurodegenerative disease
    Dennis W Dickson
    Department of Pathology Neuropathology, Mayo Clinic, Jacksonville, Florida 32224, USA
    J Clin Invest 110:1403-5. 2002
  2. pmc TREM2 in neurodegeneration: evidence for association of the p.R47H variant with frontotemporal dementia and Parkinson's disease
    Sruti Rayaprolu
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    Mol Neurodegener 8:19. 2013
  3. pmc Normal cognition in transgenic BRI2-Aβ mice
    Jungsu Kim
    Department of Neuroscience and Center for Translational Research in Neurodegenerative Disease, University of Florida, 1275 Center Drive, Gainesville, FL 32610, USA
    Mol Neurodegener 8:15. 2013
  4. pmc Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis
    Christophe Verbeeck
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    Mol Neurodegener 7:53. 2012
  5. pmc LRRK2 knockout mice have an intact dopaminergic system but display alterations in exploratory and motor co-ordination behaviors
    Kelly M Hinkle
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    Mol Neurodegener 7:25. 2012
  6. pmc Replication of EPHA1 and CD33 associations with late-onset Alzheimer's disease: a multi-centre case-control study
    Minerva M Carrasquillo
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA
    Mol Neurodegener 6:54. 2011
  7. pmc Age-related increase in amyloid plaque burden is associated with impairment in conditioned fear memory in CRND8 mouse model of amyloidosis
    Amanda Hanna
    Center for Translational Research in Neurodegenerative Disease and Department of Neuroscience, University of Florida, 1275 Center Dr, Gainesville, FL, 32610, USA
    Alzheimers Res Ther 4:21. 2012
  8. pmc Overlapping profiles of Aβ peptides in the Alzheimer's disease and pathological aging brains
    Brenda D Moore
    Center for Translational Research in Neurodegenerative Disease, Department of Neuroscience, McKnight Brain Institute, College of Medicine, University of Florida, 1275 Center Drive, Gainesville, FL, 32610, USA
    Alzheimers Res Ther 4:18. 2012
  9. pmc Glutathione S-transferase omega genes in Alzheimer and Parkinson disease risk, age-at-diagnosis and brain gene expression: an association study with mechanistic implications
    Mariet Allen
    Mayo Clinic Florida, Department of Neuroscience, Jacksonville, FL, USA
    Mol Neurodegener 7:13. 2012
  10. pmc Polymorphic genes of detoxification and mitochondrial enzymes and risk for progressive supranuclear palsy: a case control study
    Lisa F Potts
    Department of Anatomical Sciences and Neurobiology, University of Louisville, KY, USA
    BMC Med Genet 13:16. 2012

Research Grants

  1. GENETICS AND MOLECULAR BIOLOGY OF PARKINSONISM
    Dennis Dickson; Fiscal Year: 2007

Detail Information

Publications121 found, 100 shown here

  1. pmc Misfolded, protease-resistant proteins in animal models and human neurodegenerative disease
    Dennis W Dickson
    Department of Pathology Neuropathology, Mayo Clinic, Jacksonville, Florida 32224, USA
    J Clin Invest 110:1403-5. 2002
  2. pmc TREM2 in neurodegeneration: evidence for association of the p.R47H variant with frontotemporal dementia and Parkinson's disease
    Sruti Rayaprolu
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    Mol Neurodegener 8:19. 2013
    ..With this in mind we set out to assess the genetic association of the Alzheimer's disease-related risk variant in TREM2 (rs75932628, p.R47H) with other related neurodegenerative disorders...
  3. pmc Normal cognition in transgenic BRI2-Aβ mice
    Jungsu Kim
    Department of Neuroscience and Center for Translational Research in Neurodegenerative Disease, University of Florida, 1275 Center Drive, Gainesville, FL 32610, USA
    Mol Neurodegener 8:15. 2013
    ..BRI2-Aβ mice produce high levels of Aβ peptides and BRI2-Aβ1-42 mice develop amyloid pathology that is similar to the pathology observed in mutant human APP transgenic models...
  4. pmc Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis
    Christophe Verbeeck
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    Mol Neurodegener 7:53. 2012
    ..In this paper we report the development of somatic brain transgenic (SBT) mice using recombinant adeno-associated virus (rAAV) to investigate how FUS mutations lead to neurodegeneration...
  5. pmc LRRK2 knockout mice have an intact dopaminergic system but display alterations in exploratory and motor co-ordination behaviors
    Kelly M Hinkle
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    Mol Neurodegener 7:25. 2012
    ..Finally, we confirm that loss of LRRK2 caused degeneration in the kidney, accompanied by a progressive enhancement of autophagic activity and accumulation of autofluorescent material, but without evidence of biphasic changes...
  6. pmc Replication of EPHA1 and CD33 associations with late-onset Alzheimer's disease: a multi-centre case-control study
    Minerva M Carrasquillo
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA
    Mol Neurodegener 6:54. 2011
    ..abstract:..
  7. pmc Age-related increase in amyloid plaque burden is associated with impairment in conditioned fear memory in CRND8 mouse model of amyloidosis
    Amanda Hanna
    Center for Translational Research in Neurodegenerative Disease and Department of Neuroscience, University of Florida, 1275 Center Dr, Gainesville, FL, 32610, USA
    Alzheimers Res Ther 4:21. 2012
    ....
  8. pmc Overlapping profiles of Aβ peptides in the Alzheimer's disease and pathological aging brains
    Brenda D Moore
    Center for Translational Research in Neurodegenerative Disease, Department of Neuroscience, McKnight Brain Institute, College of Medicine, University of Florida, 1275 Center Drive, Gainesville, FL, 32610, USA
    Alzheimers Res Ther 4:18. 2012
    ..To attempt to distinguish between these possibilities we have systematically characterized Aβ peptides in a postmortem series of PA, AD and non-demented control (NDC) brains...
  9. pmc Glutathione S-transferase omega genes in Alzheimer and Parkinson disease risk, age-at-diagnosis and brain gene expression: an association study with mechanistic implications
    Mariet Allen
    Mayo Clinic Florida, Department of Neuroscience, Jacksonville, FL, USA
    Mol Neurodegener 7:13. 2012
    ..4,617 controls) and PD (678 PDs vs. 712 controls) for association with disease risk (case-controls), age-at-diagnosis (cases) and brain gene expression levels (autopsied subjects)...
  10. pmc Polymorphic genes of detoxification and mitochondrial enzymes and risk for progressive supranuclear palsy: a case control study
    Lisa F Potts
    Department of Anatomical Sciences and Neurobiology, University of Louisville, KY, USA
    BMC Med Genet 13:16. 2012
    ....
  11. pmc An evaluation of the impact of MAPT, SNCA and APOE on the burden of Alzheimer's and Lewy body pathology
    Christian Wider
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    J Neurol Neurosurg Psychiatry 83:424-9. 2012
    ..The study investigates the effects of genetic factors on the pathology of Alzheimer's disease (AD) and Lewy body (LB) diseases, including Parkinson's disease and dementia with Lewy bodies...
  12. pmc Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
    Ya Fei Xu
    Department of Neuroscience, Mayo Clinic, Jacksonville, 32224, USA
    Mol Neurodegener 6:73. 2011
    ..Such findings support a direct link between altered TDP-43 function and neurodegeneration...
  13. pmc Development of monoclonal antibodies and quantitative ELISAs targeting insulin-degrading enzyme
    Anthony DelleDonne
    Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road S, Jacksonville, FL 32224, USA
    Mol Neurodegener 4:39. 2009
    ..abstract:..
  14. pmc Progranulin in frontotemporal lobar degeneration and neuroinflammation
    Zeshan Ahmed
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL, USA
    J Neuroinflammation 4:7. 2007
    ....
  15. pmc Altered microRNA expression in frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations
    Jannet Kocerha
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA
    BMC Genomics 12:527. 2011
    ..Here we examine the role of miRNAs in FTLD patients with TAR DNA-binding protein 43 pathology (FTLD-TDP) caused by genetic mutations in the progranulin (PGRN) gene...
  16. pmc Hippocampal sclerosis dementia differs from hippocampal sclerosis in frontal lobe degeneration
    Catalina Amador-Ortiz
    Department of Pathology Neuropathology and Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL, USA
    Acta Neuropathol 113:245-52. 2007
    ..Corpora amylacea tended to be more frequent in HSD than in FTLD-U, but there was no difference in frequency of argyrophilic grains...
  17. doi request reprint Neuropathology of non-motor features of Parkinson disease
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Parkinsonism Relat Disord 15:S1-5. 2009
    ....
  18. pmc Neuropathology of non-Alzheimer degenerative disorders
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Int J Clin Exp Pathol 3:1-23. 2009
    ..The major neurodegenerative diseases can be classified into amyloidoses, tauopathies, alpha-synucleinopathies and TDP-43 proteinopathies...
  19. pmc Neuropathology of frontotemporal lobar degeneration-tau (FTLD-tau)
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA
    J Mol Neurosci 45:384-9. 2011
    ..The anatomical distribution of tau pathology determines the clinical presentation of PSP and CBD, as well as PiD. The basis for this selective cortical vulnerability in FTLD-tau is unknown...
  20. pmc Apoptotic mechanisms in Alzheimer neurofibrillary degeneration: cause or effect?
    Dennis W Dickson
    Department of Pathology, Mayo Clinic College of Medicine, Jacksonville, Florida 32224, USA
    J Clin Invest 114:23-7. 2004
    ..Proteolysis of tau may be critical to neurofibrillary degeneration, which correlates with dementia...
  21. pmc Common variant in GRN is a genetic risk factor for hippocampal sclerosis in the elderly
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Neurodegener Dis 7:170-4. 2010
    ..Recently, a common genetic variant in the 3' untranslated region (3'UTR) of GRN (rs5848; c.*78C>T) located in a microRNA binding site regulated progranulin expression, and the T-allele was increased in FTLD-TDP compared to controls...
  22. doi request reprint Evidence in favor of Braak staging of Parkinson's disease
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    Mov Disord 25:S78-82. 2010
    ..In this situation, LBs may be unrelated to PD and more likely related to factors inherent to AD and the selective vulnerability of the amygdala to both Alzheimer and alpha-synuclein pathologies...
  23. ncbi request reprint Required techniques and useful molecular markers in the neuropathologic diagnosis of neurodegenerative diseases
    Dennis W Dickson
    Departments of Pathology Neuropathology and Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL, 32224, USA
    Acta Neuropathol 109:14-24. 2005
    ..A diagnostic algorithm is suggested for neuropathologic evaluation of neurodegenerative disorders. The importance of clinical information is emphasized in arriving at the most precise and meaningful neuropathologic diagnosis...
  24. pmc Building a more perfect beast: APP transgenic mice with neuronal loss
    Dennis W Dickson
    Department of Pathology Neuropathology, Mayo Clinic, Jacksonville, Florida 32224, USA
    Am J Pathol 164:1143-6. 2004
  25. pmc Widespread alterations of alpha-synuclein in multiple system atrophy
    D W Dickson
    Department of Pathology, Mayo Clinic Jacksonville, Jacksonville, Florida, USA
    Am J Pathol 155:1241-51. 1999
    ..These findings provide evidence that modifications of alpha-synuclein in MSA may be more widespread than obvious histopathology. Moreover, these alterations may constitute a biochemical signature for the synucleinopathies...
  26. ncbi request reprint Multiple system atrophy: a sporadic synucleinopathy
    D W Dickson
    Department of Pathology, Mayo Clinic Jacksonville, FL 32224, USA
    Brain Pathol 9:721-32. 1999
    ..Thus, MSA and LBD are both synucleinopathies, and they may comprise different poles of a disease spectrum that includes sporadic disorders as well as genetically determined disorders such as familial Lewy body Parkinsonism...
  27. ncbi request reprint Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration
    D W Dickson
    Department of Pathology, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, USA, Email
    J Neurol 246:II6-15. 1999
    ..Further clinicopathologic studies will refine our understanding of these disorders and open the possibility that common etiologic factors may be identified for these unusual sporadic tauopathies...
  28. ncbi request reprint Neuropathology of Pick's disease
    D W Dickson
    Department of Pathology, Mayo Clinic Jacksonville, FL, USA
    Neurology 56:S16-20. 2001
    ..This is contrasted with other Pick's disease subtypes, including Constantinidis' Type B (corticobasal degeneration) and Type C (dementia lacking distinctive histology)...
  29. ncbi request reprint Alpha-synuclein and the Lewy body disorders
    D W Dickson
    Department of Pathology, Mayo Clinic, Jacksonville, Florida 32224, USA
    Curr Opin Neurol 14:423-32. 2001
    ..Whether inclusion body formation is an adaptive response or is directly related to degeneration of neuronal and glial cells is a topic of current research...
  30. doi request reprint Evidence that incidental Lewy body disease is pre-symptomatic Parkinson's disease
    Dennis W Dickson
    Neuropathology Laboratory, Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Acta Neuropathol 115:437-44. 2008
    ..The findings suggest that iLBD is preclinical PD and that the lack of symptoms is due to subthreshold pathology...
  31. pmc TDP-43 immunoreactivity in neurodegenerative disorders: disease versus mechanism specificity
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA
    Acta Neuropathol 115:147-9. 2008
  32. ncbi request reprint TDP-43 in differential diagnosis of motor neuron disorders
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL, 32224, USA
    Acta Neuropathol 114:71-9. 2007
    ..It also reveals subclinical MND in a subset of cases of FTLD without clinical or pathologic evidence of MND...
  33. ncbi request reprint Progressive supranuclear palsy: pathology and genetics
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Brain Pathol 17:74-82. 2007
    ..Imaging studies suggest that there may be sensitive and specific means to differentiate PSP from other parkinsonian disorders, but identification of a diagnostic biomarker is still elusive...
  34. pmc Linking selective vulnerability to cell death mechanisms in Parkinson's disease
    Dennis W Dickson
    Department of Pathology, Mayo Clinic College of Medicine, Jacksonville, FL, USA
    Am J Pathol 170:16-9. 2007
  35. ncbi request reprint Pick's disease: a modern approach
    D W Dickson
    Department of Research, Mayo Clinic Jacksonville, Florida 32224, USA
    Brain Pathol 8:339-54. 1998
    ..A specific molecular marker and a genetic locus for familial cases are not known...
  36. doi request reprint Neuropathology of variants of progressive supranuclear palsy
    Dennis W Dickson
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    Curr Opin Neurol 23:394-400. 2010
    ..This review highlights the range of clinical and pathologic presentations of PSP and its variants...
  37. pmc Neuropathological features of corticobasal degeneration presenting as corticobasal syndrome or Richardson syndrome
    Naomi Kouri
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Brain 134:3264-75. 2011
    ..Atrophy of anterior corpus callosum may be a potential neuroimaging marker to differentiate corticobasal degeneration from progressive supranuclear palsy in patients with Richardson syndrome...
  38. doi request reprint Progranulin gene mutation with an unusual clinical and neuropathologic presentation
    Christian Wider
    Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA
    Mov Disord 23:1168-73. 2008
    ..This study further expands the clinical and pathological spectrum of PGRN mutations, and suggests the diagnosis could be missed in some individuals with atypical presentations...
  39. ncbi request reprint Decreased neprilysin immunoreactivity in Alzheimer disease, but not in pathological aging
    Deng Shun Wang
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    J Neuropathol Exp Neurol 64:378-85. 2005
    ..The results add further evidence that PA is distinct from AD and indicate that decreased Abeta degradation by NEP is unlikely to contribute significantly to amyloid deposition in PA or, in many cases, of AD...
  40. pmc Prominent phenotypic variability associated with mutations in Progranulin
    Brendan J Kelley
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurobiol Aging 30:739-51. 2009
    ..Some kindreds with PGRN mutations exhibited lateralized topography of degeneration across all affected individuals...
  41. ncbi request reprint APOE E4 is a determinant for Alzheimer type pathology in progressive supranuclear palsy
    Yoshio Tsuboi
    Department of Neurology, Mayo Clinic, Jacksonville, FL, USA
    Neurology 60:240-5. 2003
    ..To assess demographic and genetic determinants of Alzheimer type pathology in progressive supranuclear palsy (PSP)...
  42. ncbi request reprint Neuropathologic outcome of mild cognitive impairment following progression to clinical dementia
    Gregory A Jicha
    Alzheimer s Disease Research Center and Department of Neurology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 63:674-81. 2006
    ..The pathologic outcome of patients diagnosed with mild cognitive impairment (MCI) following progression to dementia is poorly understood...
  43. ncbi request reprint Neurofilament inclusion body disease: a new proteinopathy?
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Brain 126:2291-303. 2003
    ..We propose the term neurofilament inclusion body disease for this entity...
  44. ncbi request reprint Alpha1-antichymotrypsin, an inflammatory protein overexpressed in Alzheimer's disease brain, induces tau phosphorylation in neurons
    Jaya Padmanabhan
    The Johnnie B Byrd Sr Alzheimer s Center and Research Institute Jacksonville, FL, USA
    Brain 129:3020-34. 2006
    ....
  45. ncbi request reprint Screening for neurofilament inclusion disease using alpha-internexin immunohistochemistry
    Hirotake Uchikado
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Neurology 64:1658-9. 2005
  46. ncbi request reprint Increased frequency of argyrophilic grain disease in Alzheimer disease with 4R tau-specific immunohistochemistry
    Yasuhiro Fujino
    Department of Pathology Neuropathology, Mayo Clinic College of Medicine, Jacksonville, Florida 32224, USA
    J Neuropathol Exp Neurol 64:209-14. 2005
    ..These findings suggest advanced age and possibly MAPT H1 are risk factors for AGD, even in the setting of concurrent AD, in which neurofibrillary degeneration is associated with accumulation of both 3R and 4R tau...
  47. pmc TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease
    Catalina Amador-Ortiz
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA
    Ann Neurol 61:435-45. 2007
    ....
  48. ncbi request reprint Assembly of tau in transgenic animals expressing P301L tau: alteration of phosphorylation and solubility
    Naruhiko Sahara
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    J Neurochem 83:1498-508. 2002
    ..The results suggest that tau in S2 represents an intermediate from which insoluble tau is derived, and that phosphorylation may play a role in filament formation and/or stabilization...
  49. ncbi request reprint Contribution of changes in ubiquitin and myelin basic protein to age-related cognitive decline
    Deng Shun Wang
    Department of Neuroscience and Pathology Neuropathology, Birdsall 317, Mayo Clinic Jacksonville, 4500 Sam Pablo Road, Jacksonville, FL 32224, USA
    Neurosci Res 48:93-100. 2004
    ..An age-related decrease in MBP immunoreactivity was detected in NCI cases (r=0.71). These results support the hypothesis that white matter pathology may contribute to age-associated decline in cognition...
  50. pmc Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice
    Ya Fei Xu
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    J Neurosci 30:10851-9. 2010
    ..This TDP-43 transgenic line provides a valuable tool for identifying potential roles of wild-type TDP-43 within the CNS and for studying TDP-43-associated neurotoxicity...
  51. pmc Pallidonigral TDP-43 pathology in Perry syndrome
    Christian Wider
    Department of Neurology, Mayo Clinic, Jacksonville, FL, USA
    Parkinsonism Relat Disord 15:281-6. 2009
    ..This study reports clinical, genetic and neuropathologic investigations of Perry syndrome...
  52. ncbi request reprint Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
    J Neuropathol Exp Neurol 66:142-51. 2007
    ..On the other hand, there is no histopathologic feature or combination of features that is pathognomonic. Neuronal intranuclear inclusions are virtually always present, but they can be detected in PGRN(-) cases...
  53. ncbi request reprint Argyrophilic grain disease is a sporadic 4-repeat tauopathy
    Takashi Togo
    Department of Pathology, Mayo Clinic, Jacksonville, Florida 32224, USA
    J Neuropathol Exp Neurol 61:547-56. 2002
    ..These results suggest that AGD, PSP and CBD are 4R tauopathies that share common pathologic, biochemical, and genetic characteristics...
  54. ncbi request reprint Argyrophilic grain disease: neuropathology, frequency in a dementia brain bank and lack of relationship with apolipoprotein E
    Takashi Togo
    Department of Pathology, Mayo Clinic, Jacksonville, Fla 32224, USA
    Brain Pathol 12:45-52. 2002
    ..This suggests that AGD is an independent disease process from AD...
  55. ncbi request reprint Progressive white matter pathology in the spinal cord of transgenic mice expressing mutant (P301L) human tau
    Wen Lang Lin
    Mayo Clinic College of Medicine, Jacksonville, Florida, 32224, USA
    J Neurocytol 34:397-410. 2005
    ..The progressive axonal pathology is most consistent with a dying-back process caused by abnormal accumulation of tau in upstream neurons, while vacuolar myelinopathy may be a secondary manifestation of neuroinflammation...
  56. ncbi request reprint Apoptosis in oligodendrocytes is associated with axonal degeneration in P301L tau mice
    Cindy Zehr
    Neurogenetics Laboratory, Mayo Clinic, Jacksonville, FL 32224, USA
    Neurobiol Dis 15:553-62. 2004
    ..It is unknown if loss of oligodendrocytes either through apoptosis or through the formation of intracellular tau lesions further contributes to the neurodegeneration seen in these mice...
  57. ncbi request reprint Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy
    Zeshan Ahmed
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL, USA
    Brain 131:460-72. 2008
    ..These clinical and pathological findings suggest that PSP-PNLA should be considered a variant of PSP...
  58. ncbi request reprint Dementia with Lewy bodies: neuropathology
    Dennis W Dickson
    Department of Pathology, Mayo Clinic Jacksonville, Jacksonville, Florida 32224, USA
    J Geriatr Psychiatry Neurol 15:210-6. 2002
    ..Additional clinicopathologic studies are warranted to address the role of other Lewy-related pathology, most notably Lewy neurites, in the cognitive impairment of DLB...
  59. ncbi request reprint Cognitive performance correlates with cortical isopeptide immunoreactivity as well as Alzheimer type pathology
    Deng Shun Wang
    Departments of Neuroscience and Pathology, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    J Alzheimers Dis 13:53-66. 2008
    ..Protein cross-linking and aggregation are important molecular processes in Alzheimer's disease (AD), and tissue transglutaminase (tTG) catalyzes protein cross-linking...
  60. pmc Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations
    Jennifer L Whitwell
    Division of Behavioral Neurology, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Arch Neurol 64:371-6. 2007
    ..Mutations in the progranulin gene (PGRN) have recently been identified as a cause of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) in some families...
  61. pmc Differential incorporation of tau isoforms in Alzheimer's disease
    Marisol Espinoza
    Department of Pathology, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Forchheimer 526, Bronx, NY 10461, USA
    J Alzheimers Dis 14:1-16. 2008
    ....
  62. pmc The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins
    Chad A Dickey
    Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, Florida, USA
    J Clin Invest 117:648-58. 2007
    ....
  63. ncbi request reprint Neuropathologic, biochemical, and molecular characterization of the frontotemporal dementias
    Ryan T Mott
    Department of Pathology Neuropathology, Duke University Medical Center, Durham, North Carolina 27710, USA
    J Neuropathol Exp Neurol 64:420-8. 2005
    ..We also identified a new family with the R406W mutation and pathology consistent with NFTD. This study validates the recently proposed diagnostic criteria and forms a framework for further refinement of this classification scheme...
  64. pmc Alzheimer disease-like phenotype associated with the c.154delA mutation in progranulin
    Brendan J Kelley
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 67:171-7. 2010
    ..To characterize a kindred with a familial neurodegenerative disorder associated with a mutation in progranulin (PGRN), with emphasis on the unique clinical features in this kindred...
  65. ncbi request reprint Effect of MAPT and APOE on prognosis of progressive supranuclear palsy
    Yasuhiko Baba
    Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA
    Neurosci Lett 405:116-9. 2006
    ..These results support the assertion that the H1/H1 genotype may contribute to the earlier occurrence of clinical symptoms...
  66. ncbi request reprint A presenilin 1 mutation (L420R) in a family with early onset Alzheimer disease, seizures and cotton wool plaques, but not spastic paraparesis
    Antony E Shrimpton
    Department of Pathology, SUNY Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA
    Neuropathology 27:228-32. 2007
    ..p.L420R (g.1508T > G) is the mutation responsible for EOAD, seizures and CWP without SP in this family...
  67. ncbi request reprint Ultrastructural neuronal pathology in transgenic mice expressing mutant (P301L) human tau
    Wen Lang Lin
    Mayo Clinic, Jacksonville, Florida 32224, USA
    J Neurocytol 32:1091-105. 2003
    ..These P301L transgenic mice exhibit many features common to human tauopathies, making them a valuable model to study the pathogenesis of these uncommon disorders...
  68. pmc Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Acta Neuropathol 118:349-58. 2009
    ..These findings extend previously described clinicopathological associations of FTLD-TDP subtypes and support the notion that FTLD-TDP subtypes may be distinct clinicopathologic disorders...
  69. ncbi request reprint Aging blunts ischemic-preconditioning-induced neuroprotection following transient global ischemia in rats
    Zhen He
    Department of Pharmacology, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Curr Neurovasc Res 2:365-74. 2005
    ..05), suggesting that mechanisms of ischemic cell death may change with aging. In conclusion, aging alters mechanisms of ischemic cell death in CA1 neurons and ischemic tolerance mechanisms are blunted by aging...
  70. pmc Age and apoE associations with complex pathologic features in Alzheimer's disease
    Gregory A Jicha
    Department of Neurology, Mayo Clinic, Rochester, MN, Jacksonville, FL, United States
    J Neurol Sci 273:34-9. 2008
    ....
  71. pmc Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members
    NiCole Finch
    Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Brain 132:583-91. 2009
    ..We propose that plasma GRN levels could be used as a reliable and inexpensive tool to identify all GRN mutation carriers in early-onset dementia populations and asymptomatic at-risk individuals...
  72. pmc Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis
    Nicola J Rutherford
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, United States of America
    PLoS Genet 4:e1000193. 2008
    ....
  73. pmc Leucine-rich repeat kinase 2 gene-associated disease: redefining genotype-phenotype correlation
    Christian Wider
    Department of Neurology, Mayo Clinic, Jacksonville, FL, USA
    Neurodegener Dis 7:175-9. 2010
    ..Patients carrying an LRRK2 mutation display significant variability of clinical and pathologic phenotypes across and within affected families...
  74. pmc Replication of CLU, CR1, and PICALM associations with alzheimer disease
    Minerva M Carrasquillo
    Department of Neuroscience, Mayo Clinic, 4500 San Pablo Rd, Birdsall Building, Jacksonville, FL 32224, USA
    Arch Neurol 67:961-4. 2010
    ..To test for replication of the association between variants in the CLU, CR1, and PICALM genes with Alzheimer disease...
  75. pmc TDP-43 in neurodegenerative disorders
    Casey Cook
    Mayo Clinic, 4500 San Pablo Road Jacksonville, Florida 32224, USA
    Expert Opin Biol Ther 8:969-78. 2008
    ..Biochemically, TDP-43 proteinopathies are characterized by decreased solubility, hyperphosphorylation, and cleavage of TDP-43 into 25- and 35-kDa fragments, and by altered cellular localization...
  76. ncbi request reprint Hereditary diffuse leukoencephalopathy with spheroids: clinical, pathologic and genetic studies of a new kindred
    Yasuhiko Baba
    Department of Neurology, Mayo Clinic College of Medicine, Jacksonville, FL, USA
    Acta Neuropathol 111:300-11. 2006
    ..Immunohistochemistry for APP and alphaB-crystallin demonstrates distinctive neurodegeneration in cerebral axons and perikarya...
  77. pmc Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
    Yong Jie Zhang
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Proc Natl Acad Sci U S A 106:7607-12. 2009
    ..Finally, by generating a conformation-dependent antibody that detects C-terminal fragments, we show that this toxic cleavage product is specific for pathologic inclusions in human TDP-43 proteinopathies...
  78. pmc Co-localization of glycogen synthase kinase-3 with neurofibrillary tangles and granulovacuolar degeneration in transgenic mice
    Takashi Ishizawa
    Department of Neuroscience, Mayo Clinic Jacksonville, Jacksonville, FL 32225, USA
    Am J Pathol 163:1057-67. 2003
    ....
  79. ncbi request reprint Argyrophilic grain disease in demented subjects presenting initially with amnestic mild cognitive impairment
    Gregory A Jicha
    Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA
    J Neuropathol Exp Neurol 65:602-9. 2006
    ..AGD is a common pathologic finding in subjects who have been diagnosed with amnestic MCI...
  80. ncbi request reprint Lewy bodies in progressive supranuclear palsy represent an independent disease process
    Hirotake Uchikado
    Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA
    J Neuropathol Exp Neurol 65:387-95. 2006
    ....
  81. ncbi request reprint The effect of tau genotype on clinical features in FTDP-17
    Yasuhiko Baba
    Department of Neurology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Parkinsonism Relat Disord 11:205-8. 2005
    ..7; 95% confidence interval, 1.4-98.7; P=0.008). Our results suggest that tau genotype does not influence the disease course. However, it may predispose to a specific clinical sign in the early stage of FTDP-17...
  82. ncbi request reprint Tau accumulation in astrocytes in progressive supranuclear palsy is a degenerative rather than a reactive process
    Takashi Togo
    Departments of Pathology Neuropathology and Neuroscience, Birdsall 317, Mayo Clinic, Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Acta Neuropathol 104:398-402. 2002
    ..Unlike NFT, astrocytic degeneration does not seem to contribute to gliosis or neuronal loss in PSP, and its clinical significance remains unclear...
  83. ncbi request reprint Colocalization of tau and alpha-synuclein epitopes in Lewy bodies
    Takashi Ishizawa
    Department of Pathology Neuropathology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    J Neuropathol Exp Neurol 62:389-97. 2003
    ..The present results suggest that tau may coaggregate with alpha-synuclein in LBs, especially in neuronal populations vulnerable to both NFTs and LBs...
  84. ncbi request reprint MR imaging of brainstem atrophy in progressive supranuclear palsy
    Jerzy Slowinski
    Department of Neurology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, United States
    J Neurol 255:37-44. 2008
    ..To enhance the sensitivity and specificity of the clinical diagnosis of progressive supranuclear palsy (PSP), neuroradiological parameters established in pathologically proven cases are needed...
  85. pmc Validation of the neuropathologic criteria of the third consortium for dementia with Lewy bodies for prospectively diagnosed cases
    Hiroshige Fujishiro
    Department of Pathology and Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    J Neuropathol Exp Neurol 67:649-56. 2008
    ....
  86. doi request reprint Cardiac sympathetic denervation correlates with clinical and pathologic stages of Parkinson's disease
    Hiroshige Fujishiro
    Department of Pathology Neuropathology, Mayo Clinic, Jacksonville, Florida, USA
    Mov Disord 23:1085-92. 2008
    ..42, P < 0.05). This study demonstrates that cardiac sympathetic degeneration and alpha-synuclein pathology is present in presymptomatic phase of PD, and that both increase with disease duration and severity...
  87. pmc Glucosidase-beta variations and Lewy body disorders
    Matthew J Farrer
    Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA
    Parkinsonism Relat Disord 15:414-6. 2009
    ..0 (95% CI: 0.3-29, p=0.3). All three affected carriers were classified as diffuse Lewy body disease (n=3/50; 6%). Our study suggests glucosidase-beta variants have a limited role in susceptibility to Lewy body disease in North America...
  88. pmc Abeta42 is essential for parenchymal and vascular amyloid deposition in mice
    Eileen McGowan
    Department Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA
    Neuron 47:191-9. 2005
    ..These data establish that Abeta1-42 is essential for amyloid deposition in the parenchyma and also in vessels...
  89. ncbi request reprint Hippocampal progenitor cells express nestin following cerebral ischemia in rats
    Zhen He
    Department of Pharmacology, Mayo Clinic Jacksonville, Jacksonville, Florida 32224, USA
    Neuroreport 16:1541-4. 2005
    ..eight-vessel occlusion, respectively). Confocal microscopy verified that a subset of the bromedeoxyuridine-positive cells expressed nestin. In conclusion, severe ischemia elicits nestin expression in hippocampal progenitor cells in rats...
  90. ncbi request reprint Frontotemporal dementia and parkinsonism linked to chromosome 17
    Zbigniew K Wszołek
    Department of Neurology, Mayo Clinic, 4500 San Pablo Rd, Jacksonsville, FL 32224, USA
    Folia Neuropathol 43:258-70. 2005
    ..The definitive diagnosis of FTDP-17 requires a set of clinical and pathological features combined with a molecular genetic analysis. Currently, there is no known effective treatment for FTDP-17...
  91. ncbi request reprint Rates of cerebral atrophy in autopsy-confirmed progressive supranuclear palsy
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street NW, Rochester, MN 55905, USA
    Ann Neurol 59:200-3. 2006
    ..To determine the rates of cerebral atrophy and ventricular expansion in six patients with autopsy confirmed progressive supranuclear palsy (PSP) and multiple antemortem volumetric head MRI scans...
  92. ncbi request reprint Absence of rapid eye movement sleep behavior disorder in 11 members of the pallidopontonigral degeneration kindred
    Bradley F Boeve
    Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minn 55905, USA
    Arch Neurol 63:268-72. 2006
    ..There are no reports on the possible association of rapid eye movement sleep without atonia and RBD with any familial tauopathy...
  93. pmc Overexpression of wild-type murine tau results in progressive tauopathy and neurodegeneration
    STEPHANIE J ADAMS
    Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Am J Pathol 175:1598-609. 2009
    ..This model will provide an important tool for understanding the early events leading to the development of tau pathology and a model for analysis of potential therapeutic targets for sporadic tauopathies...
  94. ncbi request reprint Correlation between antemortem magnetic resonance imaging findings and pathologically confirmed corticobasal degeneration
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 61:1881-4. 2004
    ..Various pathologic findings, including corticobasal degeneration (CBD), progressive supranuclear palsy, and frontotemporal degenerations, underlie CBS...
  95. ncbi request reprint Is the neuropathological 'gold standard' diagnosis dead? Implications of clinicopathological findings in an autosomal dominant neurodegenerative disorder
    Ryan J Uitti
    Department of Neurology and the Section of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA
    Parkinsonism Relat Disord 10:461-3. 2004
    ..We conclude that in certain circumstances genetic studies may provide definitive arbitration of validity of clinical and pathological diagnostic criteria...
  96. ncbi request reprint Apolipoprotein E epsilon 4 is a determinant for Alzheimer-type pathologic features in tauopathies, synucleinopathies, and frontotemporal degeneration
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Arch Neurol 61:1579-84. 2004
    ....
  97. ncbi request reprint Failure to wean from a ventilator caused by ANNA-1 seropositive paraneoplastic syndrome
    James F Meschia
    Department of Neurology, Mayo Clinic, Jacksonville, Florida 32224, USA
    Eur Neurol 50:112-4. 2003
  98. ncbi request reprint Dementia with Lewy bodies may present as dementia and REM sleep behavior disorder without parkinsonism or hallucinations
    Tanis J Ferman
    Department of Psychiatry and Psychology, Mayo Clinic and Foundation, Jacksonville, Florida 32224, USA
    J Int Neuropsychol Soc 8:907-14. 2002
    ..This provides further evidence in support of including RBD as one of the core diagnostic features of DLB...
  99. ncbi request reprint Clinical correlates of the pathology underlying parkinsonism: a population perspective
    James H Bower
    Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA
    Mov Disord 17:910-6. 2002
    ..Despite this selection bias, the retrospective data collection, and the small sample size, we made several observations that illustrate the difficulty in achieving an accurate antemortem diagnosis of parkinsonism...
  100. pmc Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer's disease
    Keith A Josephs
    Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA
    Mov Disord 25:1246-52. 2010
    ..In subjects presenting with CBS, prominent temporoparietal, especially posterior temporal and inferior parietal, atrophy may be a clue to the presence of underlying AD pathology...
  101. ncbi request reprint Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease
    Keith A Josephs
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Arch Neurol 63:506-12. 2006
    ..The ability to detect the clinical signs of dementia and motor neuron disease in pathologically confirmed FTLD-MND has not been assessed...

Research Grants3

  1. GENETICS AND MOLECULAR BIOLOGY OF PARKINSONISM
    Dennis Dickson; Fiscal Year: 2007
    ..abstract_text> ..