Peer-reviewed veterinary case report
Immunosuppression alters disease severity in juvenile Batten disease mice.
- Journal:
- Journal of neuroimmunology
- Year:
- 2011
- Authors:
- Seehafer, Sabrina S et al.
- Affiliation:
- University of Rochester School of Medicine & Dentistry · United States
- Species:
- rodent
Abstract
Autoantibodies to brain proteins are present in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease) patients and in the Cln3-/- mouse model of this disease, suggesting an autoimmune component to pathogenesis. Using genetic or pharmaceutical approaches to attenuate this immune response in Cln3-/- mice, we demonstrate decreased neuroinflammation, decreased deposition of immunoglobulin G in the brain and protection of vulnerable neuron populations. Moreover, immune suppression results in a significant improvement in motor performance providing for the first plausible therapeutic approach for juvenile Batten disease.
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Search related cases →Original publication: https://pubmed.ncbi.nlm.nih.gov/20937531/