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.
Original language | English |
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Pages (from-to) | 169-172 |
Number of pages | 4 |
Journal | Journal of Neuroimmunology |
Volume | 230 |
Issue number | 1-2 |
DOIs | |
State | Published - Jan 2011 |
Keywords
- Batten disease
- CLN3
- Immunosuppression
- JNCL