Abstract

The staff of the Neuropathology Core will coordinate brain autopsies, dissect and store autopsy brain tissues for research and perform brain cutting, diagnostic grading and morphometric and immunocytochemical evaluation on all autopsy cases of Huntington's disease (HD) and appropriate control cases. A new computer database will be established to record clinical and pathological data on each case. This information will be available to investigators in a manner that maintains the confidentiality of patient identification. Research tissue will be used by a number of different investigators, including those in this core and in Projects 4 and 5, and for RFLP studies of genetic linkage and presymptomatic testing families. The staff of the Neuropathology Core will devise tissue dissection protocols to supply optimally prepared tissue for the different research projects. A study of neuropathological diagnosis and histopathological grading in HD will be made, oriented toward improved diagnosis in cases with minimal pathologic changes and cases with confounding lesions such as stroke.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Program Projects (P01)
Project #
2P01NS016375-11
Application #
3861241
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
11
Fiscal Year
1991
Total Cost
Indirect Cost

  Institution

Name
Johns Hopkins University
Department
Type
DUNS #
045911138
City
Baltimore
State
MD
Country
United States
Zip Code
21218

  Publications

Faria, Andreia V; Ratnanather, J Tilak; Tward, Daniel J et al. (2016) Linking white matter and deep gray matter alterations in premanifest Huntington disease. Neuroimage Clin 11:450-460
Krause, Amanda; Mitchell, Claire; Essop, Fahmida et al. (2015) Junctophilin 3 (JPH3) expansion mutations causing Huntington disease like 2 (HDL2) are common in South African patients with African ancestry and a Huntington disease phenotype. Am J Med Genet B Neuropsychiatr Genet 168:573-85
Ross, Christopher A; Pantelyat, Alex; Kogan, Jane et al. (2014) Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction. Mov Disord 29:1351-8
Hua, Jun; Unschuld, Paul G; Margolis, Russell L et al. (2014) Elevated arteriolar cerebral blood volume in prodromal Huntington's disease. Mov Disord 29:396-401
Unschuld, Paul G; Liu, Xinyang; Shanahan, Megan et al. (2013) Prefrontal executive function associated coupling relates to Huntington's disease stage. Cortex 49:2661-73
Unschuld, Paul G; Edden, Richard A E; Carass, Aaron et al. (2012) Brain metabolite alterations and cognitive dysfunction in early Huntington's disease. Mov Disord 27:895-902
Guo, Zhihong; Rudow, Gay; Pletnikova, Olga et al. (2012) Striatal neuronal loss correlates with clinical motor impairment in Huntington's disease. Mov Disord 27:1379-86
Fu, Jinrong; Jin, Jing; Cichewicz, Robert H et al. (2012) trans-(-)-?-Viniferin increases mitochondrial sirtuin 3 (SIRT3), activates AMP-activated protein kinase (AMPK), and protects cells in models of Huntington Disease. J Biol Chem 287:24460-72
Rosenblatt, Adam; Kumar, Brahma V; Mo, Alisa et al. (2012) Age, CAG repeat length, and clinical progression in Huntington's disease. Mov Disord 27:272-6
Ratovitski, Tamara; Chighladze, Ekaterine; Arbez, Nicolas et al. (2012) Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis. Cell Cycle 11:2006-21

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