Research Topics
Genomes and GenesSpecies | Stephen J DearmondSummaryAffiliation: University of California Country: USA Publications
| Collaborators
|
Detail Information
Publications
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseasesStephen J Dearmond
Department of Pathology, University of California San Francisco, 1855 Folsom Street MCB 269, San Francisco, CA 94143 0803, USA
Mol Neurodegener 5:6. 2010..The results of this study indicate that GSI + Qa work synergistically to prevent dendrite degeneration and to block formation of PrPSc...- Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgeneCarsten Korth
Institute for Neurodegenerative Diseases and Department of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 100:4784-9. 2003..Our discovery of a previously undescribed chimeric transgene with abbreviated incubation times for sCJD prions should facilitate studies on the prion species barrier and human prion diversity... - Behavioral variant frontotemporal dementia with corticobasal degeneration pathology: phenotypic comparison to bvFTD with Pick's diseaseKatherine P Rankin
University of California San Francisco, San Francisco, CA, USA
J Mol Neurosci 45:594-608. 2011..Despite a remarkable overlap between the two patient types, bvFTD patients with underlying CBD pathology show subtle clinical features that may distinguish them from patients with Pick's disease neuropathology... - Divergent social functioning in behavioral variant frontotemporal dementia and Alzheimer disease: reciprocal networks and neuronal evolutionWilliam W Seeley
Department of Neurology, University of California at San Francisco, CA 94143 1207, USA
Alzheimer Dis Assoc Disord 21:S50-7. 2007..We suggest that the regional and neuronal vulnerability patterns seen in bvFTD and AD underlie the divergent impact of these disorders on recently evolved social-emotional functions... - Resistance of bovine spongiform encephalopathy (BSE) prions to inactivationKurt Giles
Institute for Neurodegenerative Diseases, University of California San Francisco, San Francisco, CA, USA
PLoS Pathog 4:e1000206. 2008..Our studies contend that any prion inactivation procedures must be validated by bioassay against the prion strain for which they are intended to be used... - Seizures in corticobasal degeneration: a case reportVanja C Douglas
University of California Department of Neurology Box 0114 400 Parnassus Ave, 8th Floor Neurology Clinic San Franciso, CA 94143 0114, USA
Neurocase 15:352-6. 2009..Thus, the occurrence of seizures in a patient with dementia should not exclude the diagnosis of CBD... - Discovering the mechanisms of neurodegeneration in prion diseasesStephen J Dearmond
Department of Pathology, Institute for Neurodegenerative Diseases, University of California, San Francisco, 94143 0511, USA
Neurochem Res 29:1979-98. 2004.... - Neurotoxicity of intra-arterial papaverine preserved with chlorobutanol used for the treatment of cerebral vasospasm after aneurysmal subarachnoid hemorrhageWade S Smith
Department of Neurology, University of California, San Francisco, Calif 94134 0114, USA
Stroke 35:2518-22. 2004..Papaverine is used to vasodilate cerebral arteries undergoing vasospasm from subarachnoid hemorrhage. However, papaverine inhibits cellular respiration in vitro and could cause neurotoxicity in humans... - Pathologic evidence that the T188R mutation in PRNP is associated with prion diseaseMaria Carmela Tartaglia
Memory and Aging Center, University of California, San Francisco, San Francisco, California, USA
J Neuropathol Exp Neurol 69:1220-7. 2010.... - Clinicopathological correlations in corticobasal degenerationSuzee E Lee
Memory and Aging Center, University of California San Francisco, San Francisco, CA 94143, USA
Ann Neurol 70:327-40. 2011..To characterize cognitive and behavioral features, physical findings, and brain atrophy patterns in pathology-proven corticobasal degeneration (CBD) and corticobasal syndrome (CBS) with known histopathology... - Early frontotemporal dementia targets neurons unique to apes and humansWilliam W Seeley
Memory and Aging Center, Department of Neurology, University of California, San Francisco, San Francisco, CA, USA
Ann Neurol 60:660-7. 2006..Despite progress toward understanding the genetic and molecular bases of FTD, no class of selectively vulnerable neurons has been identified... - Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob diseaseMichael D Geschwind
Department of Neurology, University of California, San Francisco Medical Center, San Francisco, CA 94143, USA
Arch Neurol 60:813-6. 2003..Previous studies have suggested that, in the proper clinical context, the 14-3-3 protein in cerebrospinal fluid is a reliable marker for sporadic CJD... - Perspectives on prion biology, prion disease pathogenesis, and pharmacologic approaches to treatmentStephen J Dearmond
Department of Pathology Neuropathology Unit, Institute for Neurodegenerative Diseases, University of California, 513 Parnassus Avenue, San Francisco, CA, USA
Clin Lab Med 23:1-41. 2003.... - Fundamentals of prion biology and diseasesStephen J Dearmond
Department of Pathology Neuropathology, HSW 430, University of California, San Francisco, CA 94143 0506, USA
Toxicology 181:9-16. 2002..Finally, the emergence of a new variant of CJD in Great Britain and to a lesser extent in Europe and its relationship to the emergence of a particularly virulent form of bovine spongiform encephalopathy will be discussed... - A change in the conformation of prions accompanies the emergence of a new prion strainDavid Peretz
Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143, USA
Neuron 34:921-32. 2002..These results suggest a causal relationship between species barriers, changes in PrP(Sc) conformation, and the emergence of new prion strains... - Transmission of elk and deer prions to transgenic miceGULTEKIN TAMGUNEY
Institute for Neurodegenerative Diseases, University of California San Francisco, 513 Parnassus Ave, San Francisco, CA 94143 0518, USA
J Virol 80:9104-14. 2006..These findings suggest that CWD prions from elk, mule deer, and white-tailed deer can be readily transmitted among these three cervid species... - A gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brainsPatricia Spilman
Departments of Pathology and Neurology and Institute for Neurodegenerative Disease, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 105:10595-600. 2008..Whether less toxic GSIs can be used in place of LY411575 to prolong survival remains to be determined... - Dominant-negative inhibition of prion replication in transgenic miceVeronique Perrier
Institute for Neurodegenerative Diseases and Departments of Pathology, Biochemistry and Biophysics, Cellular and Molecular Pharmacology, Medicine, and Neurology, University of California, San Francisco, CA 94143 0518, USA
Proc Natl Acad Sci U S A 99:13079-84. 2002..However, expression of dominant-negative PrP alone had no deleterious effects on the mice and did not support prion propagation... - Prion and doppel proteins bind to granule cells of the cerebellumGiuseppe Legname
Institute for Neurodegenerative Diseases and Departments of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 99:16285-90. 2002..Identifying the ligands to which PrP-Fc, Dpl-Fc, and dominant-negative PrP bind may provide new insights into the functions of PrPC and Dpl as well as the mechanism of PrPSc formation... - Transmission barriers for bovine, ovine, and human prions in transgenic miceMichael R Scott
Institute for Neurodegenerative Diseases, University of California, San Francisco, 94143 0518, USA
J Virol 79:5259-71. 2005..Single-amino-acid substitutions in chimeric PrP transgenes produced profound changes in incubation times that allowed us to distinguish prions causing BSE from those causing scrapie... - Design and construction of diverse mammalian prion strainsDavid W Colby
Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 106:20417-22. 2009.... - Mutant PrPSc conformers induced by a synthetic peptide and several prion strainsPatrick Tremblay
Institute for Neurodegenerative Diseases and Department of Neurology, University of California, San Francisco, California 94143, USA
J Virol 78:2088-99. 2004.... - Synthetic mammalian prionsGiuseppe Legname
Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
Science 305:673-6. 2004..Neuropathological findings suggest that a novel prion strain was created. Our results provide compelling evidence that prions are infectious proteins... - Measuring prions by bioluminescence imagingGULTEKIN TAMGUNEY
Institute for Neurodegenerative Diseases and Department of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 106:15002-6. 2009..Our studies argue that BLI is likely to be a suitable surrogate for measuring prion infectivity, and might be useful in the study of Tg mouse models for other neurodegenerative illnesses... - Variably protease-sensitive prionopathy: a new sporadic disease of the prion proteinWen Quan Zou
Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
Ann Neurol 68:162-72. 2010..The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV)... - Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypesGiuseppe Legname
Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 103:19105-10. 2006..The biophysical explanation for the unprecedented plasticity of PrP(Sc) remains to be determined... - Notch-1 activation and dendritic atrophy in prion diseaseNako Ishikura
Department of Pathology Neuropathology, Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 102:886-91. 2005..Whether diminishing Notch-1 activation in vivo can prevent or even reverse neurodegeneration in prion disease remains to be established... - Prions in skeletal musclePatrick J Bosque
Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 99:3812-7. 2002..Furthermore, muscle may provide a readily biopsied tissue from which to diagnose prion disease in asymptomatic animals and even humans... - Strain-specified characteristics of mouse synthetic prionsGiuseppe Legname
Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 102:2168-73. 2005..8 M. The incubation times, neuropathological lesion profiles, and Gdn1/2 values indicate that MoSP1 prions differ from RML and many other prion strains derived from sheep with scrapie and cattle with bovine spongiform encephalopathy... - Developmental expression of PrP in the post-implantation embryoPatrick Tremblay
Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143 0518, USA
Brain Res 1139:60-7. 2007..This pattern of Prnp expression in the nervous system appears to persist throughout the adult life of mammals... - The neurodegeneration sequence in prion diseases: evidence from functional, morphological and ultrastructural studies of the GABAergic systemEssia Bouzamondo-Bernstein
Institute for Neurodegenerative Diseases, and Department of Pathology, University of California San Francisco, San Francisco, California, USA
J Neuropathol Exp Neurol 63:882-99. 2004..In the context of these abnormalities, evoked release of [3H]-GABA from cortical and thalamic synaptosomes was significantly decreased, which correlated well with the accumulation of PrP(Sc) in synaptosomes and cell membrane fractions... - Inactivation of prions by acidic sodium dodecyl sulfateDavid Peretz
Institute for Neurodegenerative Diseases, 513 Parnassus Ave, HSE-774, San Francisco, CA 94143-0518, USA
J Virol 80:322-31. 2006..Our findings form the basis for a noncorrosive system that is suitable for inactivating prions on surgical instruments, as well as on other medical and dental equipment... - Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion proteinGULTEKIN TAMGUNEY
Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143 0518, USA
J Gen Virol 90:1035-47. 2009..The susceptibility of 'cervidized' mice to 'ovinized' prions raises the question about why CWD has not been reported in other parts of the world where cervids and scrapie-infected sheep coexist... - Transmission and detection of prions in fecesJiri G Safar
Institute for Neurodegenerative Diseases, University of California, San Francisco, San Francisco, USA
J Infect Dis 198:81-9. 2008..Our findings suggest that horizontal transmission of disease among herbivores may occur through the consumption of feces or foodstuff tainted with prions from feces of CWD-infected cervids and scrapie-infected sheep... - Correlating DWI MRI with pathologic and other features of Jakob-Creutzfeldt diseaseMichael D Geschwind
Departments of Neurology, University of California, San Francisco, UCSF Memory and Aging Center, Box 1207, SanFrancisco, CA 94143 1207, USA
Alzheimer Dis Assoc Disord 23:82-87. 2009..77; P=0.0006), followed by reactive astrocytic gliosis (r=0.63, P=0.008). This case provides further evidence that DWI abnormalities correlate well with the clinical features and with PrP(Sc) accumulation and vacuolation... - Prion clearance in bigenic miceJiri G Safar
Institute for Neurodegenerative Diseases, University of California, 513 Parnassus Ave, San Francisco, CA 94143, USA
J Gen Virol 86:2913-23. 2005..Moreover, these bigenic mice make it possible to determine both components of PrP(Sc) accumulation, i.e. the rates of formation and clearance, for various strains of prions exhibiting different incubation times... - Diagnosis of human prion diseaseJiri G Safar
Institute for Neurodegenerative Diseases, Memory and Aging Center, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 102:3501-6. 2005..Moreover, IHC should not be used as the standard against which all other immunodiagnostic techniques are compared because an immunoassay, such as the CDI, is substantially more sensitive... - Human prions and plasma lipoproteinsJiri G Safar
Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143, USA
Proc Natl Acad Sci U S A 103:11312-7. 2006..Whether detection of PrP(Sc) in VLDL and LDL particles can be adapted into an antemortem diagnostic test for prions in the blood of humans, livestock, and free-ranging cervids remains to be determined... - Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissuesGerold Schmitt-Ulms
Institute for Neurodegenerative Disease, San Francisco, California 94143, USA
Nat Biotechnol 22:724-31. 2004..Many of these proteins have been implicated in cell adhesion/neuritic outgrowth, and harbor immunoglobulin C2 and fibronectin type III-like motifs... - Human growth hormone-related iatrogenic Creutzfeldt-Jakob disease with abnormal imagingAaron M Lewis
Department of Neurology, University of California, San Francisco, USA
Arch Neurol 63:288-90. 2006..As symptoms referable to the cerebellum occur early in iCJD, it suggests that these MRI sequences may allow earlier diagnosis of this form of prion disease...