The Neuropathology Core plays a critical role in this Program Project by providing detailed histopathological characterization of a large number of animal and human tissues, utilizing several techniques.
In Specific Aim 1, the Core has the task of determining the presence, type, severity and anatomic distribution of the structural lesions, along with the topography and pattern of deposition of the scrapie prion protein (PrPSc) in brains from mice and hamsters. Brains will be sectioned according to precise and fixed coordinates and processed for histological and immunohistochemical examinations. If required, other tissues will be similarly processed. Highly specialized services such as lesion profiling and PET blot and histoblot will also be provided. Altogether, these techniques allow for the establishment of the histopathological phenotype, and are designed to assure that data can be compared.
Specific Aim 2 is directed at acquiring and characterizing brain tissues from human prion diseases needed for the coordinated studies of Research Projects 1 and 3. The Core will take advantage of the unique opportunity of acquiring the rare cases needed from the tissue collection of the National Prion Disease Pathology Surveillance Center (NPDPSC), led by the Core Director. The third objective of the Core is to report the results of tissue examinations to the Project Leaders and store processed tissue samples, which will remain available to the members of the Program Project. The qualifications of the Core Director, the variety and consistency of the examinations and access to a unique tissue collection make the Neuropathology Core essential to the success of the entire Program Project. Project

Agency
National Institute of Health (NIH)
Institute
National Institute of Allergy and Infectious Diseases (NIAID)
Type
Research Program Projects (P01)
Project #
1P01AI106705-01A1
Application #
8779084
Study Section
Special Emphasis Panel (ZAI1-RWM-M (S3))
Project Start
Project End
Budget Start
2014-07-01
Budget End
2015-06-30
Support Year
1
Fiscal Year
2014
Total Cost
$104,429
Indirect Cost
$27,154
Name
Case Western Reserve University
Department
Type
DUNS #
077758407
City
Cleveland
State
OH
Country
United States
Zip Code
44106
Theint, Theint; Nadaud, Philippe S; Surewicz, Krystyna et al. (2016) (13)C and (15)N chemical shift assignments of mammalian Y145Stop prion protein amyloid fibrils. Biomol NMR Assign :
Abskharon, Romany; Wang, Fei; Vander Stel, Kayla J et al. (2016) The role of the unusual threonine string in the conversion of prion protein. Sci Rep 6:38877
Choi, Jin-Kyu; Cali, Ignazio; Surewicz, Krystyna et al. (2016) Amyloid fibrils from the N-terminal prion protein fragment are infectious. Proc Natl Acad Sci U S A 113:13851-13856
Hu, Ping Ping; Morales, Rodrigo; Duran-Aniotz, Claudia et al. (2016) Role of Prion Replication in the Strain-dependent Brain Regional Distribution of Prions. J Biol Chem 291:12880-7
Pirisinu, Laura; Di Bari, Michele A; D'Agostino, Claudia et al. (2016) Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases. Sci Rep 6:20443
Orrú, Christina D; Groveman, Bradley R; Raymond, Lynne D et al. (2015) Bank Vole Prion Protein As an Apparently Universal Substrate for RT-QuIC-Based Detection and Discrimination of Prion Strains. PLoS Pathog 11:e1004983
Cali, Ignazio; Miller, Cathleen J; Parisi, Joseph E et al. (2015) Distinct pathological phenotypes of Creutzfeldt-Jakob disease in recipients of prion-contaminated growth hormone. Acta Neuropathol Commun 3:37