The major goals of Core C are to support the research activities of this PPG by obtaining, characterizing, and distributing postmortem brain tissues from patients with MSA. The Core will provide detailed neuropathological data from these cases to Core and Project investigators, as well as provide advice and technical support to investigators in this PPG. These goals will be accomplished by the following specific aims: 1. To perform brain autopsies on PPG participants with clinical features of MSA in a timely fashion and according to standard protocol. 2. To provide detailed neuropathologic evaluation to establish a definitive diagnosis and to document the presence or absence of other central (CNS) and peripheral (PNS) nervous system pathologies. Neuropathologic data for all brains will be collected using standardized methods for gross dissection and neurohistology. These approaches include hematoxylin and eosin (H&E), myelin and silver stains, and The Consortium to Establish a Registry for Alzheimer's Disease (CERAD) and Braak scoring, in all cases. Additionally we will immunostain tissues with antibodies to a-synuclein. In cases with Lewy bodies (LB), LBs will be counted in cortical regions to assign a diagnostic category according to the Consortium for Dementia with Lewy Bodies criteria. We will assess the distribution and abundance of GCIs and related LB-like neuronal inclusions, dystrophic synuclein neurites, and other synuclein pathologies in the CNS and PNS of patients with MSA, compared to controls without MSA or any other neurological disease. 3. Collect cerebral spinal fluid (CSF), blood (huffy coat), and blood-spot samples on all cases. CSF and huffy coat samples will be frozen and banked for future research studies. 4. Store brain tissue and CSF, blood (buffy coat), and blood spot samples and provide pathologically well-characterized tissue samples for on-going research (Projects 2, 3, and Core D). 5. Monitor the acquisition and distribution of these tissue samples using a computerized database and maintain a computerized archive of histopathologic findings for research use by investigators in this PPG. 6. Provide advice and technical support to facilitate studies by PPG investigators on synucleinopathies and synucleinopathy models, especially Projects 2 and 3. 6. Prepare and store DNA from MSA patients.
Rockenstein, Edward; Ostroff, Gary; Dikengil, Fusun et al. (2018) Combined Active Humoral and Cellular Immunization Approaches for the Treatment of Synucleinopathies. J Neurosci 38:1000-1014 |
Coon, Elizabeth A; Ahlskog, J Eric; Silber, Michael H et al. (2018) Do selective serotonin reuptake inhibitors improve survival in multiple system atrophy? Parkinsonism Relat Disord 48:51-53 |
Ogaki, Kotaro; Martens, Yuka A; Heckman, Michael G et al. (2018) Multiple system atrophy and apolipoprotein E. Mov Disord 33:647-650 |
Wenning, Gregor; Trojanowski, John Q; Kaufmann, Horacio et al. (2018) Is multiple system atrophy an infectious disease? Ann Neurol 83:10-12 |
Cutsforth-Gregory, Jeremy K; McKeon, Andrew; Coon, Elizabeth A et al. (2018) Ganglionic Antibody Level as a Predictor of Severity of Autonomic Failure. Mayo Clin Proc 93:1440-1447 |
Singer, Wolfgang; Berini, Sarah E; Sandroni, Paola et al. (2017) Pure autonomic failure: Predictors of conversion to clinical CNS involvement. Neurology 88:1129-1136 |
Valera, Elvira; Spencer, Brian; Mott, Jennifer et al. (2017) MicroRNA-101 Modulates Autophagy and Oligodendroglial Alpha-Synuclein Accumulation in Multiple System Atrophy. Front Mol Neurosci 10:329 |
Valera, Elvira; Spencer, Brian; Fields, Jerel A et al. (2017) Combination of alpha-synuclein immunotherapy with anti-inflammatory treatment in a transgenic mouse model of multiple system atrophy. Acta Neuropathol Commun 5:2 |
Coon, Elizabeth A; Fealey, Robert D; Sletten, David M et al. (2017) Anhidrosis in multiple system atrophy involves pre- and postganglionic sudomotor dysfunction. Mov Disord 32:397-404 |
El-Agnaf, Omar; Overk, Cassia; Rockenstein, Edward et al. (2017) Differential effects of immunotherapy with antibodies targeting ?-synuclein oligomers and fibrils in a transgenic model of synucleinopathy. Neurobiol Dis 104:85-96 |
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