期刊
JOURNAL OF NEUROIMMUNOLOGY
卷 230, 期 1-2, 页码 169-172出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.jneuroim.2010.08.024
关键词
CLN3; JNCL; Batten disease; Immunosuppression
资金
- National Institutes of Health [NS40580, NS44310, NS41930]
- Beat Batten Foundation
- Wellcome Trust [GR079491MA]
- European Commission [LSHM-CT-2003-503051]
- Batten Disease Support and Research Association
- Natalie Fund
- Batten Disease Family Association
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS044310, R21NS040580, R21NS041930] Funding Source: NIH RePORTER
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. (C) 2010 Elsevier B.V. All rights reserved.
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