Peter St George Hyslop

Summary

Affiliation: University of Toronto
Country: Canada

Publications

  1. pmc Role of p73 in Alzheimer disease: lack of association in mouse models or in human cohorts
    Badri Vardarajan
    Department of Medicine Biomedical Genetics, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118, USA
    Mol Neurodegener 8:10. 2013
  2. pmc The isotropic fractionator provides evidence for differential loss of hippocampal neurons in two mouse models of Alzheimer's disease
    Hannah Brautigam
    Fishberg Department of Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA
    Mol Neurodegener 7:58. 2012
  3. pmc Both common variations and rare non-synonymous substitutions and small insertion/deletions in CLU are associated with increased Alzheimer risk
    Karolien Bettens
    Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, 2610 Antwerpen, Belgium
    Mol Neurodegener 7:3. 2012
  4. doi request reprint Assembly of the presenilin γ-/ε-secretase complex
    P St George-Hyslop
    Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Ontario, Canada
    J Neurochem 120:84-8. 2012
  5. pmc Acute dosing of latrepirdine (Dimebon), a possible Alzheimer therapeutic, elevates extracellular amyloid-beta levels in vitro and in vivo
    John W Steele
    Departments of Neurology, Psychiatry and Alzheimer s Disease Research Center, Mount Sinai School of Medicine, New York, NY, 10029, USA
    Mol Neurodegener 4:51. 2009
  6. pmc Dietary composition modulates brain mass and solubilizable Abeta levels in a mouse model of aggressive Alzheimer's amyloid pathology
    Steve Pedrini
    Farber Institute for the Neurosciences, Jefferson Medical College, Philadelphia PA USA
    Mol Neurodegener 4:40. 2009
  7. pmc Analysis of IFT74 as a candidate gene for chromosome 9p-linked ALS-FTD
    Parastoo Momeni
    Laboratory of Neurogenetics, National Institute of Aging, NIH, Bethesda, MD, USA
    BMC Neurol 6:44. 2006
  8. ncbi request reprint Molecular biology and genetics of Alzheimer's disease
    Peter H St George-Hyslop
    Department of Medicine, Division of Neurology, The Toronto Hospital, University of Toronto, 6, Queen s Park Crescent West, Toronto, Ontario, Canada
    C R Biol 328:119-30. 2005
  9. ncbi request reprint Genetic factors in the genesis of Alzheimer's disease
    P H St George-Hyslop
    Department of Medicine Division of Neurology, University Health Network, University of Toronto, Toronto, Ontario, Canada M5S 3H2
    Ann N Y Acad Sci 924:1-7. 2000
  10. ncbi request reprint The presenilin 1 protein is a component of a high molecular weight intracellular complex that contains beta-catenin
    G Yu
    Centre for Research in Neurodegenerative Diseases, Departments of Medicine and Medical Biophysics, University of Toronto, Toronto, Ontario M5S 3H2, Canada
    J Biol Chem 273:16470-5. 1998

Collaborators

Detail Information

Publications26

  1. pmc Role of p73 in Alzheimer disease: lack of association in mouse models or in human cohorts
    Badri Vardarajan
    Department of Medicine Biomedical Genetics, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118, USA
    Mol Neurodegener 8:10. 2013
    ..Here, we reevaluated tau pathologies in the same TgCRND8 mouse model as the previous studies...
  2. pmc The isotropic fractionator provides evidence for differential loss of hippocampal neurons in two mouse models of Alzheimer's disease
    Hannah Brautigam
    Fishberg Department of Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA
    Mol Neurodegener 7:58. 2012
    ..We investigated whether two APP mouse models characterized by different folding states of amyloid showed different neuronal densities using an accurate method of cell counting...
  3. pmc Both common variations and rare non-synonymous substitutions and small insertion/deletions in CLU are associated with increased Alzheimer risk
    Karolien Bettens
    Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, 2610 Antwerpen, Belgium
    Mol Neurodegener 7:3. 2012
    ....
  4. doi request reprint Assembly of the presenilin γ-/ε-secretase complex
    P St George-Hyslop
    Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Ontario, Canada
    J Neurochem 120:84-8. 2012
    ..Because the structure and assembly of these complexes underlies their activity, this review will discuss current work on the assembly of the complex and on presenilin-interacting proteins that regulate secretase activity...
  5. pmc Acute dosing of latrepirdine (Dimebon), a possible Alzheimer therapeutic, elevates extracellular amyloid-beta levels in vitro and in vivo
    John W Steele
    Departments of Neurology, Psychiatry and Alzheimer s Disease Research Center, Mount Sinai School of Medicine, New York, NY, 10029, USA
    Mol Neurodegener 4:51. 2009
    ..In the current study, we assessed the effect of acute dosing of latrepirdine on levels of extracellular Abeta using in vitro and in vivo experimental systems...
  6. pmc Dietary composition modulates brain mass and solubilizable Abeta levels in a mouse model of aggressive Alzheimer's amyloid pathology
    Steve Pedrini
    Farber Institute for the Neurosciences, Jefferson Medical College, Philadelphia PA USA
    Mol Neurodegener 4:40. 2009
    ..abstract:..
  7. pmc Analysis of IFT74 as a candidate gene for chromosome 9p-linked ALS-FTD
    Parastoo Momeni
    Laboratory of Neurogenetics, National Institute of Aging, NIH, Bethesda, MD, USA
    BMC Neurol 6:44. 2006
    ..A new locus for amyotrophic lateral sclerosis--frontotemporal dementia (ALS-FTD) has recently been ascribed to chromosome 9p...
  8. ncbi request reprint Molecular biology and genetics of Alzheimer's disease
    Peter H St George-Hyslop
    Department of Medicine, Division of Neurology, The Toronto Hospital, University of Toronto, 6, Queen s Park Crescent West, Toronto, Ontario, Canada
    C R Biol 328:119-30. 2005
    ..Knowledge of this biochemical cascade now provides several potential targets for the development of diagnostics and therapeutics...
  9. ncbi request reprint Genetic factors in the genesis of Alzheimer's disease
    P H St George-Hyslop
    Department of Medicine Division of Neurology, University Health Network, University of Toronto, Toronto, Ontario, Canada M5S 3H2
    Ann N Y Acad Sci 924:1-7. 2000
    ..Some of the genes are highly penetrant (PS1, PS2, beta APP); the other, APOE, is a weaker susceptibility factor. Several additional genes are suspected to exist but have not yet been cloned...
  10. ncbi request reprint The presenilin 1 protein is a component of a high molecular weight intracellular complex that contains beta-catenin
    G Yu
    Centre for Research in Neurodegenerative Diseases, Departments of Medicine and Medical Biophysics, University of Toronto, Toronto, Ontario M5S 3H2, Canada
    J Biol Chem 273:16470-5. 1998
    ....
  11. ncbi request reprint Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease
    R Sherrington
    Department of Medicine Neurology, University of Toronto, Ontario, Canada
    Nature 375:754-60. 1995
    ..Because these changes occurred in conserved domains of this gene, and are not present in normal controls, they are likely to be causative of AD3...
  12. ncbi request reprint Nicastrin binds to membrane-tethered Notch
    F Chen
    Centre for Research in Neurodegenerative Diseases Departments of Medicine and Medical Biophysics, University of Toronto, Tanz Neuroscience Building, 6 Queen s Park Crescent West, Toronto, Ontario M5S 3H2, Canada
    Nat Cell Biol 3:751-4. 2001
    ..In addition, we report that the Notch and betaAPP pathways do not significantly compete with each other...
  13. ncbi request reprint Biology of presenilins as causative molecules for Alzheimer disease
    M Nishimura
    Centre for Research in Neurodegenerative Diseases, Department of Medicine, University of Toronto, Ontario, Canada
    Clin Genet 55:219-25. 1999
    ..Furthermore, presenilins interact with beta-catenin to form presenilin complexes and presenilin mutations effect beta-catenin signalling pathways...
  14. ncbi request reprint Identification of the genetic locus for keratosis palmaris et plantaris on chromosome 17 near the RARA and keratin type I genes
    E I Rogaev
    Department of Medicine, University of Toronto, Ontario, Canada
    Nat Genet 5:158-62. 1993
    ..Our analysis provide a region which should be investigated for contiguous gene syndromes in such pedigrees...
  15. ncbi request reprint Absence of linkage between familial amyotrophic lateral sclerosis and copper chaperone for the superoxide dismutase gene locus in two Italian pedigrees
    A Orlacchio
    Centre for Research in Neurodegenerative Diseases, Tanz Neuroscience Building, University of Toronto, 6 Queen s Park Crescent West, Toronto, Ontario, Canada
    Neurosci Lett 285:83-6. 2000
    ..Diagnoses were made using the El Escorial criteria. The results of our study provide no evidence of a linkage between markers flanking the CCS gene and familial amyotrophic lateral sclerosis (FALS) in these FALS kindreds...
  16. ncbi request reprint PS1 Alzheimer's disease family with spastic paraplegia: the search for a gene modifier
    E Rogaeva
    Centre for Research in Neurodegenerative Diseases, University of Toronto, and Department of Medicine Division of Neurology, Ontario, Canada
    Neurology 61:1005-7. 2003
    ..The authors have excluded that this modifier effect originates from coding sequence variations in three SP genes or from a second mutation in the other AD genes...
  17. ncbi request reprint A novel Leu171Pro mutation in presenilin-1 gene in a Mexican family with early onset Alzheimer disease
    M G Ramírez-Dueñas
    Departamento de Fisiologia, Universidad de Guadalajara, Jalisco, Mexico
    Ann Genet 41:149-53. 1998
    ..The pedigree analysis and the literature data strongly suggest an etiopathogenic relationship of the mutation with the disorder...
  18. ncbi request reprint The Glu318Gly mutation of the presenilin-1 gene does not necessarily cause Alzheimer's disease
    K M Mattila
    Karolinska Institute, Alzheimer s Disease Research Centre, KFC, Novum, Huddinge, Sweden
    Ann Neurol 44:965-7. 1998
    ..We also found the Glu318Gly mutation in 4 healthy aged controls (range, 74-87 years). We thus conclude that the mutation is most likely a rare polymorphism not related to AD...
  19. ncbi request reprint Aberrant splicing in the presenilin-1 intron 4 mutation causes presenile Alzheimer's disease by increased Abeta42 secretion
    C De Jonghe
    Department of Molecular Genetics, Flanders Interuniversity Institute for Biotechnology VIB, Laboratory of Molecular Genetics, Born Bunge Foundation BBS, University of Antwerp UIA, Antwerpen, Belgium
    Hum Mol Genet 8:1529-40. 1999
    ..Our data indicate that in the case of intron 4 mutation, the AD pathophysiology results from the presence of the PSEN1 T113-114ins protein comparable with cases carrying dominant PSEN1 missense mutations...
  20. ncbi request reprint Inhibiting amyloid precursor protein C-terminal cleavage promotes an interaction with presenilin 1
    G Verdile
    Sir James McCusker Alzheimer s Disease Research Unit and Department of Surgery, University of Western Australia, Hollywood Private Hospital, Nedlands, Western Australia 6009
    J Biol Chem 275:20794-8. 2000
    ..These findings suggest that PS1 may mediate the shuttling of APP fragments and/or facilitate their presentation for gamma-secretase cleavage through a direct interaction...
  21. ncbi request reprint Estimation of the genetic contribution of presenilin-1 and -2 mutations in a population-based study of presenile Alzheimer disease
    M Cruts
    Laboratory of Neurogenetics, Flanders Interuniversity Institute for Biotechnology VIB, Born Bunge Foundation BBS, University of Antwerp UIA, Department of Biochemistry, Antwerpen, Belgium
    Hum Mol Genet 7:43-51. 1998
    ..Further, polymorphisms were detected in the promoter and the 5'-non-coding region of PS -1 and in intronic and exonic sequences of PS -2 that will be useful in genetic association studies...
  22. ncbi request reprint Three different mutations of presenilin 1 gene in early-onset Alzheimer's disease families
    K Kamino
    Department of Geriatric Medicine, Osaka University Medical School, Japan
    Neurosci Lett 208:195-8. 1996
    ..Our results suggested the existence of a variety of PS-1 mutations, and that early-onset FAD with PS-1 mutations is highly penetrant and is only rarely subject to modulation by genetic or environmental modifying factors...
  23. ncbi request reprint A new SPG4 mutation in a variant form of spastic paraplegia with congenital arachnoid cysts
    A Orlacchio
    Laboratory of Neurogenetics, University of Rome Tor Vergata, Rome, Italy
    Neurology 62:1875-8. 2004
    ..A molecular study has revealed a novel missense mutation, T614I, in exon 17 of SPG4, which may play a role in both focal cortical dysgenesis and neurodegeneration of the motor neurons in the corticospinal tract...
  24. ncbi request reprint Association between presenilin-1 Glu318Gly mutation and familial Alzheimer's disease in the Australian population
    K Taddei
    Sir James McCusker Alzheimer s Disease Research Unit and University of Western Australia, Department of Surgery, Hollywood Private Hospital, Nedlands, Perth 6009, Australia
    Mol Psychiatry 7:776-81. 2002
    ..0%) or the Spanish population (5.3%). These findings show that the frequency of the Glu318Gly mutation is increased in FAD patients, suggesting a potential role as a genetic risk factor contributing to the pathogenesis of familial AD...
  25. 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
    ....
  26. ncbi request reprint Variable clinical symptoms in familial amyotrophic lateral sclerosis with a novel point mutation in the Cu/Zn superoxide dismutase gene
    M Ikeda
    Department of Neurology, Gunma University School of Medicine, Maebashi, Japan
    Neurology 45:2038-42. 1995
    ..These results suggest that FALS from this novel I104F mutation shows considerable clinical variation...