IVIG Delays Onset in a Mouse Model of Gerstmann-Sträussler-Scheinker Disease
| Autor: | Martin R. Farlow, Junyi Liu, Timothy Zhu, Hongjun Peng, Gang Zhao, James A. Mastrianni, Yansheng Du, Huiying Gu, Yvonne Kirchhein, Eileen Du, Richard Dodel |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Genetically modified mouse medicine.medical_specialty Neurology Neuroscience (miscellaneous) Apoptosis Mice Transgenic Plaque Amyloid Kaplan-Meier Estimate Prion Proteins Article Pathogenesis 03 medical and health sciences Cellular and Molecular Neuroscience 0302 clinical medicine In vivo hemic and lymphatic diseases Medicine Animals Gerstmann-Straussler-Scheinker Disease Neurons biology business.industry Autoantibody Neurotoxicity Immunoglobulins Intravenous medicine.disease In vitro Disease Models Animal 030104 developmental biology Phenotype Immunology biology.protein Disease Progression Antibody business Neuroglia 030217 neurology & neurosurgery |
| Zdroj: | Molecular neurobiology. 56(4) |
| ISSN: | 1559-1182 |
| Popis: | Our previous studies showed that intravenous immunoglobulin (IVIG) contained anti-Aβ autoantibodies that might be able to treat Alzheimer’s disease (AD). Recently, we identified and characterized naturally occurring autoantibodies against PrP from IVIG. Although autoantibodies in IVIG blocked PrP fibril formation and PrP neurotoxicity in vitro, it remained unknown whether IVIG could reduce amyloid plaque pathology in vivo and be used to effectively treat animals with prion diseases. In this study, we used Gerstmann-Straussler-Scheinker (GSS)-Tg (PrP-A116V) transgenic mice to test IVIG efficacy since amyloid plaque formation played an important role in GSS pathogenesis. Here, we provided strong evidence that demonstrates how IVIG could significantly delay disease onset, elongate survival, and improve clinical phenotype in Tg (PrP-A116V) mice. Additionally, in treated animals, IVIG could markedly inhibit PrP amyloid plaque formation and attenuate neuronal apoptosis at the age of 120 days in mice. Our results indicate that IVIG may be a potential, effective therapeutic treatment for GSS and other prion diseases. |
| Databáze: | OpenAIRE |
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