JamesAMastrianniMDPhD

PubMed

• A case report of a patient with primary familial brain calcification with agenetic variant.
  Jamal Al Ali, Jessica Yang, Matthew S Phillips, Joseph Fink, James Mastrianni, Kaitlin Seibert> ;Frontiers in Neurology. 2023 Jan 1
• Challenging Cases of Neurocognitive Disorders.
  James A Mastrianni, Kaitlin Seibert> ;Seminars in Neurology. 2022 Dec 1
• 3 citations
  A NovelVariant (Lys694del) Presenting With Corticobasal Syndrome in a Family With FTD-ALS Spectrum Diseases: Case Report.
  Kaitlin Seibert, Heather Smith, Allison Lapins, Peter Pytel, James A Mastrianni> ;Frontiers in Neurology. 2022 Jan 1
• 14 citations
  Generation of human chronic wasting disease in transgenic mice.
  Zerui Wang, Kefeng Qin, Manuel V. Camacho, Ignazio Cali, Jue Yuan, Pingping Shen, Justin J. Greenlee, Qingzhong Kong, James A. Mastrianni, Wen-Quan Zou> ;Acta Neuropathologica Communications. 2021 Sep 26
• An atypical presentation of primary central nervous system lymphoma: A case report.
  Carlen Yuen, James A. Mastrianni, Saad Ali, Peter Pytel, Deric M. Park, Kourosh Rezania> ;Medicine. 2020 Sep 18
• 2 citations
  A novel PRNP-G131R variant associated with familial prion disease.
  Jumana T. Alshaikh, Kefeng Qin, Lili Zhao, James A. Mastrianni> ;Neurology. Genetics. 2020 Aug 1
• 5 citations
  “Dual Disease” TgAD/GSS mice exhibit enhanced Alzheimer’s disease pathology and reveal PrP C -dependent secretion of Aβ
  Kefeng Qin, Lili Zhao, Crystal Gregory, Ani Solanki, James A. Mastrianni> ;Scientific Reports. 2019 Jun 12
• 2 citations
  Anle138b prevents PrP plaque accumulation in Tg(PrP-A116V) mice but does not mitigate clinical disease.
  Kefeng Qin, Lili Zhao, Ani Solanki, Crystal Busch, James A. Mastrianni> ;The Journal of General Virology. 2019 May 2
• 5 citations
  IVIG Delays Onset in a Mouse Model of Gerstmann-Sträussler-Scheinker Disease
  Huiying Gu, Yvonne Kirchhein, Timothy Zhu, Gang Zhao, Hongjun Peng, Eileen Du, Junyi Liu, James A. Mastrianni, Martin R. Farlow, Richard Dodel, Yansheng Du> ;Molecular Neurobiology. 2019 Apr 1
• 6 citations
  Impaired transmissibility of atypical prions from genetic CJDG114V
  Ignazio Cali, Fadi Mikhail, Kefeng Qin, Crystal Gregory, Ani Solanki, Manuel Camacho Martinez, Lili Zhao, Brian S. Appleby, Pierluigi Gambetti, Eric M. Norstrom, James...> ;Neurology. Genetics. 2018 Aug 1
• 50 citations
  Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356
  Daniel J. Klionsky, Kotb Abdelmohsen, Akihisa Abe, Joynal Abedin, Hagai Abeliovich, Abraham Acevedo Arozena, Hiroaki Adachi, Christopher M. Adams, Peter D. Adams, Khos...> ;Autophagy. 2016 Jan 1
• 673 citations
  Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).
  Vyas, J. M., Daniel J Klionsky, Kotb Abdelmohsen, Akihisa Abe, Md Joynal Abedin, Hagai Abeliovich, Abraham Acevedo Arozena, Hiroaki Adachi, Christopher M Adams, Peter ...> ;Autophagy. 2016 Jan 1
• 13 citations
  Early Delivery of Misfolded PrP from ER to Lysosomes by Autophagy.
  Constanza J. Cortes, Kefeng Qin, Eric M. Norstrom, William N. Green, Vytautas P. Bindokas, James A. Mastrianni> ;International Journal of Cell Biology. 2013 Dec 17
• 23 citations
  In vivo parahippocampal white matter pathology as a biomarker of disease progression to Alzheimer's disease.
  Ana Solodkin, E. Elinor Chen, Gary W. Van Hoesen, Lennart Heimer, Ahmed D. Shereen, Frithjof Kruggel, James A. Mastrianni> ;The Journal of Comparative Neurology. 2013 Dec 15
• 7 citations
  Systemic transthyretin amyloidosis in a patient with bent spine syndrome
  Kourosh Rezania, Peter Pytel, Laurel J. Smit, James A. Mastrianni, Michelle A. Dina, W. Edward Highsmith, Ahmet Dogan> ;Amyloid. 2013 May 22
• 2 citations
  Acute encephalopathy as the initial manifestation of CADASIL
  Yuan Fan, Shanele McGowan, Helene Rubeiz, Robert L. Wollmann, Adil Javed, James A. Mastrianni> ;Neurology. Clinical Practice. 2012 Dec 1
• 78 citations
  Rapamycin Delays Disease Onset and Prevents PrP Plaque Deposition in a Mouse Model of Gerstmann–Sträussler–Scheinker Disease
  Constanza J. Cortes, Kefeng Qin, Julie Cook, Ani Solanki, James A. Mastrianni> ;The Journal of Neuroscience. 2012 Sep 5
• 13 citations
  Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices
  Cadman Leggett, Daniel S. McGehee, James A. Mastrianni, Wenbin Yang, Tao Bai, James R. Brorson> ;Journal of the Neurological Sciences. 2012 Jun 15
• 59 citations
  Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype.
  Suman Jayadev, David Nochlin, Parvoneh Poorkaj, Ellen J. Steinbart, James A. Mastrianni, Thomas J. Montine, Bernardino Ghetti, Gerard D. Schellenberg, Thomas D. Bird, ...> ;Annals of Neurology. 2011 Apr 1
• 82 citations
  Generation of a New Form of Human PrPSc in Vitro by Interspecies Transmission from Cervid Prions
  Marcelo A. Barria, Glenn C. Telling, Pierluigi Gambetti, James A. Mastrianni, Claudio Soto> ;The Journal of Biological Chemistry. 2011 Mar 4
• 85 citations
  The Prion Diseases
  Khalilah Brown, James A. Mastrianni> ;Journal of Geriatric Psychiatry and Neurology. 2010 Oct 11
• 158 citations
  Variably protease‐sensitive prionopathy: A new sporadic disease of the prion protein
  Wen-Quan Zou, Gianfranco Puoti, Xiangzhu Xiao, Jue Yuan, Liuting Qing, Ignazio Cali, Miyuki Shimoji, Jan P. M. Langeveld, Rudy J. Castellani, Silvio Notari, Barbara J....> ;Annals of Neurology. 2010 Aug 1
• 10 citations
  Live Cell Fluorescence Resonance Energy Transfer Predicts an Altered Molecular Association of Heterologous PrPSc with PrPC
  Suparna Mallik, Wenbin Yang, Eric M. Norstrom, James A. Mastrianni> ;The Journal of Biological Chemistry. 2010 Mar 19
• 87 citations
  The genetics of prion diseases
  James A. Mastrianni> ;Genetics in Medicine. 2010 Apr 1
• 45 citations
  A New Transgenic Mouse Model of Gerstmann–Sträussler–Scheinker Syndrome Caused by the A117V Mutation of PRNP
  Wenbin Yang, Julie Cook, Benjamin Rassbach, Azucena Lemus, Stephen J. DeArmond, James A. Mastrianni> ;The Journal of Neuroscience. 2009 Aug 12
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Journal Articles

• In vivo parahippocampal white matter pathology as a biomarker of disease progression to Alzheimer disease  
  Solodkin A, Eelinor C, Van Hoesen GW, Lennart H, Ahmed S, Frithjof K, Mastrianni J, J Comp Neurol, 7/10/2013
• Systemic transthyretin amyloidosis in a patient with bent spine syndrome. Amyloid  
  Rezania K, Pytel P, Smit LJ, Mastrianni J, Dina MA, Highsmith WE, Dogan A, 6/1/2013
• Acute encephalopathy as the initial manifestation of CADASIL  
  Fan, Y., McGowan, S., Rubeiz, H., Wollmann, R., Javed, A., Mastrianni, J., Neurology, Clinical Practice, 12/1/2012
• Rapamycin Delays Disease Onset and Prevents PrP Plaque Deposition in a Mouse Model of Gerstmann-Sträussler-Scheinker disease  
  Cortes C, Qin K, Cook J, Solanki A, and Mastrianni JA, J. Neurosci, 9/5/2012
• Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices  
  Leggett C, McGehee DS, Mastrianni J, Yang W, Bai T, Brorson JR, J. Neurol. Sci., 6/15/2012
• Rapidly Progressive Dementia does Not Always Mean Creutzfeldt-Jakob Disease  
  Mastrianni, JA, Journal Watch Neurology, 7/19/2011
• Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype  
  Jayadev, S., Nochlin, D., Poorkaj, P., Steinbart, E.J., Mastrianni, J.A., Montine, T.J., Ghetti, B., Schellenberg, G.D., Bird, T.D., Leverenz, J.B., Annals of Neurology, 4/1/2011
• Generation of a New Form of Human PrPSc in vitro by Interspecies Transmission from Cervid Prions  
  Barria M.A., Telling G.C., Gambetti P., Mastrianni J.A., Soto C., J Biol Chem, 1/1/2011
• The Prion Diseases and Related Disorders  
  Mastrianni, JA and Brown, K, The Journal of Geriatric Psychiatry and Neurology, 12/1/2010
• Protease-Sensitive Prionopathy: A New Sporadic Disease of the Prion Protein  
  Zou W., Puoti G., Xiao X, Yuan J., Qing L., Cali I., Shimoji M., Langeveld J., Castellani R., Notari S., Crain B., Schmidt R., Geschwind M., DeArmond S., Cairns N., Di..., Annals of Neurology, 8/1/2010
• Genetic Prion Diseases  
  Mastrianni, J, Genetics in Medicine, 4/1/2010
• Live Cell FRET Predicts an Altered Molecular Association of Heterologous PrPSc with PrPC  
  Mallik S., Yang W., Norstrom E.M., and Mastrianni J.A., Journal of Biological Chemistry, 1/1/2010
• A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker syndrome due to the A117V Mutation of PRNP  
  Yang W., Cook, J., Rassbach B., Lemus A., DeArmond S.J., and Mastrianni J.A., Journal of Neuroscience, 1/1/2009
• A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease  
  Gambetti, P., Dong, Z., Yuan, J., Xiao, X., Zheng, M., Alshekhlee, A., Castellani, R., Cohen, M., Barria, M.A., Gonzalez-Romero, D., Belay, E.D., Schonberger, L.B., Ma..., Annals of Neurology, 1/1/2008
• A Novel Variant of Gerstmann-Straussler-Scheinker Disease due to a P105S mutation of PRNP  
  Tunnell E., DeArmond S, Wollman R, Malik S, and Mastrianni JA, Neurology, 1/1/2008
• Identifying key components of the PrPC-PrPSc replicative interface  
  Abalos G.C., Cruite J.T., Bellon A., Hemmers S, Akagi J., Mastrianni J.A., Williamson R. A. and Solforosi, L., Journal of Biological Chemistry, 1/1/2008
• Cytosolic Prion Protein Toxicity is Independent of Cellular Prion Protein Expression and Prion Propagation  
  Norstrom E.M., Ciaccio M.F., Rassbach B., Wollman R., and Mastrianni J.A., Journal of Virology, 1/1/2007
• The charge structure of helix 1 in prion protein regulates conversion to pathogenic PrPSc  
  Norstrom E.M. and Mastrianni JA, Journal of Virology, National Microbiology Newsletter, 1/1/2006
• PRNP heterozygosity linked to primary progressive aphasia  
  Mastrianni, JA and Mesulam, M, Annals of Neurology, 1/1/2006
• The AGAAAAGA palindrome in PrP is required to generate a productive PrPSc-PrPC complex that leads to prion propagation  
  Norstrom E.M. and Mastrianni JA, Journal of Biological Chemistry, 7/22/2005
• Attacking the Elusive Prion  
  Liebman S and Mastrianni J, Trends in Molecular Medicine, 1/1/2005
• The Family that Couldn’t Sleep  
  Mastrianni, J., Neurology Today, 1/1/2005
• The Prion Diseases  
  Mastrianni, J, Clinical Neuroscience Research, 1/1/2004
• Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene  
  Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J, Parchi P, Gambetti P, Will R, Ironside J, Heinrich C, Tremblay P, DeArmond SJ, Prusiner SB, Proceedings of the National Academy of Science, 4/8/2003
• Wrinkles and folds of the prion protein  
  Mastrianni, J., and Roos, R., Neurology, 1/1/2003
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Books/Book Chapters

• The Molecular and Genetic Basis of Neurologic and Psychiatric Disease 
  Chapter: Prion Diseases 
  Mastrianni, J.A.Editors: Rosenberg, R. N. ed. DiMauro, S., Paulson, H.L., Ptacek, L., and Nestler, E.J..Lippinccott, Williams, & Wilkins2007
• Current Therapy in Neurologic Disease 
  Chapter: The Prion Diseases 
  Page 319 - 321Mastrianni, J.A.Editors: Johnson, Griffin, and McArthur.Elsevier, Philadelphia2006
• Handbook of Dementing Illnesses 
  Chapter: Prion Diseases and Dementia 
  Mastrianni, J.A.Editors: Morris, Galvin, and Holtzman.Taylor and Francis Group LLCEdition Seco,2006
• New Advances in Neurogenetics 
  Chapter: The Genetics of Prion Diseases 
  Baek, W. and Mastrianni, JA.Editors: Lynch, D..Dekker Inc.2006
• Neurological Therapeutics 
  Chapter: Prion Diseases: Transmissible Spongiform Encephalopathies 
  Mastrianni, J.A.Editors: J Noseworthy.Taylor & Francis Group2005
• Principles of Neurological Infectious Diseases 
  Chapter: Prion Diseases 
  Johnston, E.K. and Mastrianni, J.A.Editors: K. Roos.McGraw-Hill2005
• Emerging Neurological Infections 
  Chapter: Prion Diseases and Dementia 
  Page 77 - 113Mastrianni, J.A.Editors: Power and Johnson.Taylor and Francis Group, Boca Raton2005
• Regulatory Roles of Opioid Peptides 
  Chapter: Endorphinergic Neurons in the Brainstem and Central Cardiovascular Regulation 
  Kunos, G., R. Mosqueda-Garcia and J.A. MastrianniEditors: C. Farsang, P.Illes and I. Pataki.VCH Publishers, Weinheim, Deerfield Beach1987
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Abstracts/Posters

• Prion Protein Codon 129 Genotype Distribution is Altered in Primary Progressive Aphasia
  Li X, Rowland L, Mitsumoto H, Przedborski S, Bird T, Schellenberg G, Peskind E, Johnson N, Siddique T, Mesulam M-M, Weintraub S, and Mastrianni J, Annals of Neurology, NIH Press Release, Neurology Today, 1/1/2005

Lectures

• Defining the Molecular and Cell Biological Mechanisms of Human Prion Disease 
  University of Alabama Birmingham, Center for Neurophysiology and Experimental Therapeutics, Birmingh - 2/7/2013
• RNAi in Neurodegenerative Disease 
  University of Alabama Birmingham, Department of Neurobiology, Birmingham, AL - 2/7/2013
• Cognitive Neurology Rounds 
  1/1/2013
• Clinical Phenotypes and Basic Mechanisms of Prion Disease 
  Wayne State University School of Medicine, Department of Neurology - 12/1/2012
• Reducing the Risk of Cognitive Impairment 
  11/1/2012
• Dementia Update 
  11/1/2012
• Maybe Your Brain is Trying to Tell You Something 
  7/1/2012
• Our Aging Parents and Alzheimer’s: Tips for Care Giving and the Caregiver 
  East Bank Club, Chicago, IL - 3/1/2012
• The Phenotypic Diversity of Sporadic and Genetic Prion Diseases 
  Chicago, IL - 5/1/2011
• Roos Rounds 
  1/1/2011
• The Prion Diseases, A Review 
  University of Illinois at Chicago, Department of Neurology, Chicago, IL - 10/1/2010
• Of Mice and Men and Yeast – Modeling Prion Biology 
  University of California Irvine, Institute for Memory Impairment and Neurological Disorders (MIND) - 9/1/2010
• Modeling and Defining the Molecular Determinants of Prion Propagation 
  Evanston Hospital - 5/1/2010
• Molecular Determinants of Prion Propagation 
  University of Alabama Birmingham, Department of Neurology and Neurobiology - 4/1/2010
• Genetic and Transmissible Prion Diseases 
  Georgetown University School of Medicine, D.C. - 2/1/2010
• Inherited and Infectious Prion Diseases 
  Harvard University Medical School, Kuwait City, Kuwait - 11/1/2009
• Modeling Prion Disease 
  Northwestern University Depts. of Physiology and Neurology, Chicago, IL - 11/1/2008
• Developing Models of Prion Disease 
  Northwestern University Dept. of Neurology, Chicago, IL - 5/1/2008
• Alzheimer’s Disease, The Past and Future 
  Resurrection Health Care/Westlake Consortium - 4/1/2008
• The Biology of Prions and Related Diseases 
  Enrico Fermi Laboratory, Batavia, IL - 3/1/2008
• Expanding the Range of Prion-Related Diseases 
  Northwestern University, Chicago, IL., Dept. of Neurology - 1/1/2007
• The Nature of Prion Polymorphisms in Disease 
  Northwestern University, Chicago, IL - 9/1/2006
• Don’t Forget Your Folks: Memory and Aging 
  8/1/2006
• HIV and Prion Disease 
  American Academy of Neurology - 4/1/2006
• Mad Cow and Related TSE Diseases: Science, Risks and Public Policy Conference 
  University of Connecticut, Dodd Research Center, Storr, CT - 10/1/2005
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Other

• Prion Diseases 
  Mastrianni J, GeneReviews at GeneTests: Medical Genetics Information Resource
  http://www.genetests.org
  1/1/2013
• Protein Folding 
  Mastrianni, J.A., "Odyssey" program, National Public Radio, Chicago
  3/1/2001
• Alzheimer’s Disease expert, the basis of Alzheimer's Disease and current research advances 
  Mastrianni, J.A., KTSF-TV, San Francisco
  1/1/1994