Curriculum Vitae

Transmissible Spongiform Encephalopathies Advisory Committee

NAME: James A. Mastrianni, M.D., Ph.D.

POSITION TITLE: Associate Professor of Neurology

EDUCATION/TRAINING

INSTITUTION AND LOCATION	DEGREE (if applicable)	YEAR(s)	FIELD OF STUDY
Union University, Albany, NY	B.S.	----	Pharmacy
University of North Carolina, Greenville, NC	Ph.D.	----	Pharmacology
McGill University, Montreal, Canada	M.D.C.M.	-----	Medicine
University of Pennsylvania, Philadelphia, PA	Intern/Resident	1993	Neurology
University of California, San Francisco, CA	Postdoctoral	1998	Mol.Genetics/Prions

Professional Experience:

1985 - 1989: Post Doctoral Fellow - (part time while attending medical school), in the laboratory of George Kunos, M.D., Ph.D., Dept. of Pharmacology, McGill University, Canada

1988 - 1990: Staff Fellow - (part time), at the National Institute on Alcohol Abuse and Alcoholism, NIH, Rockville, MD - George Kunos, M.D., Ph.D., Laboratory Chief.

1989 - 1990: Medical Intern - Hospital of the University of Pennsylvania, Philadelphia, PA.

1990 - 1993: Resident in Neurology - Hospital of the University of Pennsylvania, Philadelphia, PA.

1993 - 1996: Postdoctoral Scholar - University of California San Francisco, CA. Funded by a fellowship award from Howard Hughes Medical Institute, Stanley B. Prusiner, M.D., mentor.

1994 - 1998: Clinical instructor in Neurology - University of California San Francisco, CA, Dr. Stephen Hauser, Chairman.

1994 - 1998: Director,Memory and Cognitive Disorders Clinic at University of California, San Francisco, CA.

1996 - 1998: Medical Director, Alzheimer's Disease Diagnostic and Treatment Center (ADDTC) at the University of California, San Francisco, CA.

1998 - 1998: Adjunct Assistant Professor of Neurology - University of California San Francisco, CA.

1998 - : Assistant Professor of Neurology - The University of Chicago Pritzker School of Medicine.

1999 - : Co-director, Center for Comprehensive Care and Research on Memory Disorders, U. of Chicago

2000 - : Committees of Microbiology and Neurobiology, The University of Chicago

2005- Associate Professor of Neurology, U. of Chicago Pritzker School of Medicine

2005- Committee on Cell Physiology

2005: Medical Advisory Board, the Schweppe Foundation

2006: Committee on Molecular Genetics and Cell Biology, The University of Chicago

2006: United States FDA-Transmissible Spongiform Encephalopathy Advisory Committee Member

Honors and Awards:

1980: Rho Chi National Pharmaceutical Honor Society.
Associate Dean's Prize for scholastic excellence in undergraduate study.
Who's Who in American Colleges and Universities

1986/7: "Prix d'Excellence" (presented by the Quebec Health Research Foundation)

1989: "Lange Medical Book Prize" for "outstanding accomplishment during medical training".

1992/3: "Zeritsky Award", for research conducted during the Neurology Residency program at HUP.

1993: Howard Hughes Medical Institute Postdoctoral Fellowship Award, "Molecular Characterization of the Phenotypic Presentations of Prion Disease".

1997: "Teaching Award nomination in Pharmacology" - for a lectures to the Pharmacy school class regarding the pharmacologic management of Alzheimer's Disease.

1998: Teaching Award - "Outstanding Clinical Preceptorship" - in the Introduction to Clinical Medicine course at UCSF School of Medicine.

1998: Brain Research Foundation - Seed grant for "Molecular Determinants of Human Prion Disease and its Relationship to ALS and Alzheimer disease".

1999: Howard Hughes Medical Institute Research Resources Grant - For Development of a Prion Disease Research Program at the University of Chicago.

1999: Schweppe Foundation - career development award.

2001: United States Food and Drug Administration (FDA), Transmissible Spongiform Encephalopathy Advisory Committee.

2001 American Federation of Aging Research-career development award.

2001: Alzheimer's Association - New Investigator Research Grant.

2002: Teacher of the Year - Department of Psychiatry at the University of Chicago

2002: National Institutes of Health - Committee for establishment of a Prion Disease Repository.

2003: Alschuler Scholar- by the Brain Research Foundation, for research in Alzheimer related disease

2003: Institute of Medicine- invited reviewer of directives outlined by the Institute for prion research strategies

2003: Public Health Service, United Kingdom- ad hoc grant reviewer

2004: Best Graduate Student Poster, Eric Norstrom (Ph.D. student in lab), Neuroscience Day, U.Chicago

2005: Siemens "Outstanding Mentor", of a high school student in the Siemens Westinghouse Competition in Math, Science, and Technology.

2006: Ginsburg Prize in Physiology, awarded to mentored graduate student (Eric Norstrom) for "best thesis" in Neurobiology at The University of Chicago, 2005.

2007: Neuroscience Award, American Academy of Neurology, by Mahesh Vidula, student in lab for paper to AAN.

Selected Publications (of ~40):

Spudich, S., Mastrianni, J.A., Gabizon, R., Kahana, E., and S. B. Prusiner: Complete Penetrance of the E200K Mutations of the Prion Protein Gene in a Population of Libyan Jews, Human Mutation Vol. 1 (6), 607-613, 1995.
Telling, G. Scott, M., Mastrianni, J., Gabizon, R., Torchia, M., Cohen, F., DeArmond, S., and Prusiner, S.: Prion Propagation in Mice Expressing Human and Chimeric PrP Transgenes Implicates the Interaction of Cellular PrP with Another Protein, Cell 83(1): 79-90, 1995.
Mastrianni, J.A., Curtis, M.T., Oberholtzer, J.C., Prusiner, S.B., and J.Y. Garbern: Ataxic Gerstmann-Straussler-Scheinker Diseased Caused by a Prion Protein Gene Mutation at Codon 117, Neurology 45(11), pp.2042 - 2050, 1995.
Mastrianni, J.A.. Iannocola, C., Myers, R., and S.B.Prusiner: Mutation of the Prion Protein Gene at Codon 208 in Familial Creutzfeldt-Jakob disease, Neurology 47(4), pp. 1305-1312, 1996.
Telling, G., Parchi, P., DeArmond, S., Cortelli, P., Montagna, P., Gabizon, R., Mastrianni, J., Lugaresi, E., Gambetti, P., and Prusiner, S.: Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity, Science 274, pp:2079-2082, December 20, 1996.
Schuff, N., Amend, D., Ezekiel, F., Steinman, S.K., Tanabe, J., Norman, D., Jagust, W., Kramer, J.H., Mastrianni, J.A., Fein, G., and Weiner, M.W.: Changes of Hippocampal N-Acetyl Aspartate and Volume in Alzheimer's Disease: A Proton MR Spectroscopic Imaging and MRI Study, Neurology 49(6), 1513-21, 1997.
Hegde, R.S., Mastrianni, J.A., Scott, M.R., DeFea, K.A., Tremblay, P., Torchia, M., DeArmond, S., Prusiner, S.B., and Lingappa, V.R.: A Transmembrane Form of the Prion Protein in Neurodegenerative Disease, Science,.6, 279(5352): 827-34, Feb., 1998.
Mastrianni, J.A., Nixon, R., Layzer, R., DeArmond, S., and Prusiner, S.B., Prion Protein Conformation in a Patient with Sporadic Fatal Insomnia, N Eng J Med., May 27;340(21):1630-8, 1999.
Worrall, B.B., Rowland L.P., Chin, S.S., and Mastrianni, J.A.: Neurological Reviews: Amyotrophy in Prion Diseases, Archives of Neurology, vol. 57,pp.33-38, 2000.
Mastrianni, J. A., Capellari, S., Glenn C. Telling, G. C., Han, D., Bosque, P., DeArmond, S. J. & Prusiner, S.B. Inherited prion disease caused by the V210I mutation: Transmission to transgenic mice, Neurology 2001 Dec 26;57(12):2198-205.
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.Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A. Apr 8, 2003.
Norstrom E.M. and Mastrianni JA. The AGAAAAGA palindrome in PrP is required to generate a productive PrP^Sc-PrP^C complex that leads to prion propagation, Journal of Biological Chemistry, Jul 22;280(29):27236-43, 2005.
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. Prion Protein Codon 129 Genotype Prevalence is Altered in Primary Progressive Aphasia, Annals of Neurology;58:858-864, 2005.
Liebman S and Mastrianni J. Attacking the Elusive Prion, Trends in Molecular Medicine, Volume 11, Issue 10, 439-441, 2005.
Norstrom E.M. and Mastrianni JA. The charge structure of helix 1 in prion protein regulates conversion to pathogenic PrP^Sc, Journal of Virology, 80(17):.8521-8529, 2006.
Norstrom E.M., Ciaccio M.F., Rassbach B., Wollman R., and Mastrianni J.A.. Cytosolic Prion Protein Toxicity is Independent of Cellular Prion Protein Expression and Prion Propagation. Journal of Virology, 81(6); 2831-2837, 2007.

Ongoing Research Support:

R01- NS046037-04
NIH (MASTRIANNI) 03/15/03-02/28/08
"Defining the prion domain of PrP"
This project studies the role of the putative prion domain segment of prion protein in the conversion of PrP C to PrP Sc and in the generation of the species barrier.
Role: PI

R01- NS051480-05
NIH (MASTRIANNI) 12/05 - 11/30/10
"Role of the Lysosome in ER associated degradation of PrP".
This project studies a proposed degradative pathway leading from the ER directly to lysosomes for the elimination of misfolded prion protein.
Role: PI

Completed Research Support:

American Federation of Aging Research (MASTRIANNI)
The Paul Beeson Physician's Faculty Scholar Award 06/01/01-05/31/04
"Aging and the Misfolded Protein: Prion Disease as a Model for Disaggregating the Aggregate".
This work focuses on the using the prion protein as a model to study protein aggregation in yeast and animals, and testing novel inhibitory peptides against protein aggregation.
Role: PI

Alzheimer's Association (MASTRIANNI) 07/01/01-06/30/03
New Investigator Research Grant
"Understanding and Inhibiting the Aggregation of Prion Protein"
This project was to develop a yeast expression system to study prion protein aggregation.
Role: PI

Schweppe Foundation (MASTRIANNI) 12/01/99 - 05/31/03
"The Role of Residue 129 of the Prion Protein as a Determinant of Prion Transmission and Susceptibility to Neurodegenerative Disease".
This project study the polymorphic 129 codon as a susceptibility factor in prion disease and other neurodegenerative diseases.
Role: PI

K08 NS01913 (MASTRIANNI)
NIH 09/01/96 - 08/31/02
"Molecular Determinants of Human Prion Diseases"
The purpose of this project was to screen for mutations of the prion protein gene, define phenotype-genotype correlations and study the transmissible nature of different familial strains of prion disease.
Role: PI