The University of Chicago Header Logo

Search Result Details

This page shows the details of why an item matched the keywords from your search.
One or more keywords matched the following properties of Mastrianni, James A.
keywords Prion Diseases
overview James Mastrianni, MD, PhD, is an expert in memory disorders and other neurodegenerative conditions. Working on a team of geriatricians, neurologists and neuropsychologists, Dr. Mastrianni provides diagnostic evaluation and long-term management of patients with Alzheimer's disease and related dementias. In a special clinic, he also evaluates patients referred to him with atypical dementias, which are unusual dementias for which a clear diagnosis cannot yet be determined. In addition to providing effective care for these patients, Dr. Mastrianni and his colleagues conduct clinical and basic science research to better understand and treat neurodegenerative diseases. Dr. Mastrianni's research, which has received funding by the National Institutes of Health (NIH), the Howard Hughes Medical Institute, the Alzheimer’s Association, among many others, focuses on understanding animal and human prion diseases. These are progressive neurodegenerative disorders that are also transmissible. They include Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, fatal insomnia, and mad cow disease, among others. Prion diseases share many similarities with more common neurodegenerative conditions, such as Alzheimer's disease, Parkinson’s disease, and Amyotrophic Lateral Sclerosis (ALS). Thus, Dr. Mastrianni’s studies that are aimed at understanding and correcting the underlying defect in prion diseases help researchers to better understand these more common diseases, as well. In fact, Dr. Mastrianni’s recent research has identified a direct link between prion disease and Alzheimer’s disease. This is a major focus of study, as is developing a gene therapy against prion disease. Dr. Mastrianni is an accomplished author who has published numerous scientific articles, book chapters and reviews. He is an invited contributor for Neurology, Journal Watch Neurology, Genetics in Medicine, Trends in Molecular Medicine, and other prestigious medical journals. Dr. Mastrianni is the recipient of many awards and honors. Most recently, he was named the Helen M. McLoraine Neuroscience Investigator of the Brain Research Foundation and was recently awarded the Frederic A. Gibbs Discovery Award for scientific achievement. He has been invited as an expert reviewer for the ECRI Institute of the U.S. Agency for Health Care Research and Quality and he has served on the Illinois Chapter of the Medical and Scientific Advisory panel for the Alzheimer’s Association and on a neuroscience scientific advisory committee of the U.S. Food and Drug Administrations (FDA). Also a dedicated educator, Dr. Mastrianni teaches and mentors students, residents, and doctoral and post-doctoral students.
One or more keywords matched the following items that are connected to Mastrianni, James A.
Item TypeName
Academic Article Prion protein conformation in a patient with sporadic fatal insomnia.
Academic Article The prion diseases: Creutzfeldt-Jakob, Gerstmann-Sträussler-Scheinker, and related disorders.
Academic Article A transmembrane form of the prion protein in neurodegenerative disease.
Academic Article Amyotrophy in prion diseases.
Academic Article The prion diseases.
Academic Article Inherited prion disease caused by the V210I mutation: transmission to transgenic mice.
Academic Article Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene.
Academic Article "Out, damned spot! out, I say!...": issues related to prion decontamination.
Academic Article Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype.
Academic Article Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein.
Academic Article The prion diseases.
Academic Article Prion disease (PrP-A117V) presenting with ataxia instead of dementia.
Academic Article Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein.
Academic Article A novel human disease with abnormal prion protein sensitive to protease.
Academic Article Anle138b prevents PrP plaque accumulation in Tg(PrP-A116V) mice but does not mitigate clinical disease.
Academic Article A novel PRNP-G131R variant associated with familial prion disease.
Search Criteria
  • Prion Diseases