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CNS expression of glucocerebrosidase corrects α-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy
Open accessAbstract
Emerging genetic and clinical evidence suggests a link between Gaucher disease and the synucleinopathies Parkinson disease and dementia with Lewy bodies. Here, we provide evidence that a mouse model of Gaucher disease ( Gba1 D409V/D409V ) exhibits characteristics of synucleinopathies, including progressive accumulation of proteinase K-resistant α-synuclein/ubiquitin aggregates in hippocampal neurons and a coincident memory deficit. Analysis of homozygous ( Gba1 D409V/D409V ) and heterozygous ( Gba1 D409V/+ and Gba1 +/− ) Gaucher mice indicated that these pathologies are a result of the combination of a loss of glucocerebrosidase activity and a toxic gain-of-function resulting from expression of the mutant enzyme. Importantly, adeno-associated virus-mediated expression of exogenous glucocerebrosidase injected into the hippocampus of Gba1 D409V/D409V mice ameliorated both the histopathological and memory aberrations. The data support the contention that mutations in GBA1 can cause Parkinson disease-like α-synuclein pathology, and that rescuing brain glucocerebrosidase activity might represent a therapeutic strategy for GBA1 -associated synucleinopathies.
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Multicenter Analysis of Glucocerebrosidase Mutations in Parkinson's Disease
Sidransky, E., Nalls, M. A., Aasly, J. O., Aharon-Peretz, J., Annesi, G., Barbosa, E. R., Bar-Shira, A., Berg, D., Bras, J., Brice, A., et al. (2009) · New England Journal of Medicine · doi:10.1056/nejmoa0901281 · Cited by 2,135
Neuroinflammation in Parkinson’s disease: A target for neuroprotection?
Hirsch, E. C., & Hunot, S. (2009) · The Lancet Neurology · doi:10.1016/s1474-4422(09)70062-6 · Cited by 1,984
Mutations in the Glucocerebrosidase Gene and Parkinson's Disease in Ashkenazi Jews
Aharon-Peretz, J., Rosenbaum, H., & Gershoni-Baruch, R. (2004) · New England Journal of Medicine · doi:10.1056/nejmoa033277 · Cited by 597
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Murphy, K. E., Gysbers, A. M., Abbott, S. K., Tayebi, N., Kim, W. S., Sidransky, E., Cooper, A., Garner, B., & Halliday, G. M. (2014) · Brain · doi:10.1093/brain/awt367 · Cited by 475
The Autophagy–Lysosomal Pathway in Neurodegeneration: A TFEB Perspective
Martini-Stoica, H., Xu, Y., Ballabio, A., & Zheng, H. (2016) · Trends in Neurosciences · doi:10.1016/j.tins.2016.02.002 · Cited by 436
Excessive burden of lysosomal storage disorder gene variants in Parkinson’s disease
Robak, L. A., Jansen, I. E., van Rooij, J., Uitterlinden, A. G., Kraaij, R., Jankovic, J., Heutink, P., Shulman, J. M., International Parkinson’s Disease Genomics Consortium (IPDGC), Nalls, M. A., et al. (2017) · Brain · doi:10.1093/brain/awx285 · Cited by 433
Gut-seeded α-synuclein fibrils promote gut dysfunction and brain pathology specifically in aged mice
Challis, C., Hori, A., Sampson, T. R., Yoo, B. B., Challis, R. C., Hamilton, A. M., Mazmanian, S. K., Volpicelli-Daley, L. A., & Gradinaru, V. (2020) · Nature Neuroscience · doi:10.1038/s41593-020-0589-7 · Cited by 405
Metadata
- Authors
- Sardi, S. P., Clarke, J., Kinnecom, C., Tamsett, T. J., Li, L., Stanek, L. M., Passini, M. A., Grabowski, G. A., Schlossmacher, M. G., Sidman, R. L., Cheng, S. H., & Shihabuddin, L. S.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2011-07-05
- Volume
- 108
- Issue
- 29
- Pages
- 12101-12106
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W1982224533
- Cited by
- 319
- References
- 4
- FWCI
- 11.17
- Type
- journal article
- Language
- en
- Open access
- green
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- Sardi, S. P., Clarke, J., Kinnecom, C., Tamsett, T. J., Li, L., Stanek, L. M., Passini, M. A., Grabowski, G. A., Schlossmacher, M. G., Sidman, R. L., Cheng, S. H., & Shihabuddin, L. S. (2011). CNS expression of glucocerebrosidase corrects α-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.1108197108
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
